Professional Documents
Culture Documents
CONTENTS
Preface
Andrew T. Parsa and Mitchel S. Berger
Epidemiology and Pathology of Intraventricular Tumors
James S. Waldron and Tarik Tihan
ix
469
Intraventricular Neurocytomas
Janet Lee, Susan M. Chang, Michael W. McDermott, and Andrew T. Parsa
483
Central neurocytomas (CNCs) are World Health Organization II benign central nervous
system (CNS) neoplasms first described in 1982 by Hassoun and his colleagues. Hallmark features of CNC include (1) occurrence in the lateral ventricle of young adults,
(2) a well-circumscribed isodense to hyperdense mass with contrast enhancement on
CT and isointense to hyperintense compared with normal brain parenchyma on T1and T2-weighted MRI, (3) resemblance to oligodendroglioma on light microscopy, (4)
neuronal origin seen in electron microscopy and immunohistochemistry, and (5) favorable prognosis with benign biologic behavior. CNCs comprise 0.1% to 0.5% of all CNS
neoplasms based on pathologic review at several neurosurgery centers. A populationbased incidence has not been established, in part because of the paucity of cases. Given
its recent distinction as a unique tumor and its low incidence, most reports of CNC are
from the pathologic literature with little data regarding its management. Furthermore,
many early cases of CNC were misdiagnosed, and treatment was based on the presumed
diagnosis of oligodendroglioma or ependymoma. Accordingly, this article presents a
comprehensive review of the literature and proposes a management paradigm for the
treatment of CNC.
VOLUME 14
509
Tumors of the lateral ventricles comprise a relatively rare heterogeneous group of lesions
in children and adults. They arise from the ependyma and subependyma that line the
ventricles, from the choroid plexus arachnoid and epithelium, or from ectopic tissue
rests that have become trapped within the ventricle or its lining. Although the lateral
ventricles are among the most surgically inaccessible areas of the brain, numerous operative approaches to the ventricles have been developed. This article first discusses the
clinical manifestations and differential diagnosis of lateral ventricular tumors. Relevant
regional anatomy and general operative strategies for these lesions are then discussed,
with particular focus on the following approaches: frontal, temporal, and parietal transcortical approaches and anterior and posterior interhemispheric approaches.
527
Advanced microsurgical techniques combined with improved neuroanesthetic and postoperative critical care have made aggressive surgical resection a mainstay in the management of posterior third ventricular and pineal region tumors. Although a variety of
approaches to the posterior third ventricle have been designed, three are in common
use. The infratentorial-supracerebellar approach takes advantage of a natural corridor
between the cerebellum and the tentorium. Supratentorial approaches include the
interhemispheric-transcallosal and occipital-transtentorial approaches. Refinements in
surgical technique have led to a more favorable outlook for patients with these uncommon tumors.
547
Intraventricular Meningiomas
Michael W. McDermott
559
Meningiomas arising in the ventricular system are rare; yet, when they do present
clinically, they are often large, most often within the atrium, and most frequently on
the left. For all these reasons, they are tumors for which it is difficult to achieve the
perfect surgical result: complete removal of a benign tumor without complications
or new neurologic morbidity. With a thorough understanding of the anatomy of structures around the ventricle, selection of the proper surgical approach, and use of modern neurosurgical techniques, however, modern-day surgical results should be superior
to those of the past.
vi
CONTENTS
Intraventricular Gliomas
Aaron S. Dumont, Elana Farace, David Schiff, and Mark E. Shaffrey
571
Significant progress has been realized in the contemporary understanding and treatment
of intraventricular gliomas. However, there remains a substantial need for continued advancement in the clinical management of patients harboring these lesions, particularly
ependymomas. This article addresses the specific types of intraventricular gliomas with
emphasis on each tumors defining characteristics and the specific nuances of management in each variant.
593
607
Intraventricular congenital lesions and colloid cysts comprise a rather large spectrum of
different pathologic conditions. In most cases, treatment in not warranted unless there is
progressive ventricular obstruction with hydrocephalus or growth of the lesion itself,
making tissue biopsy and histopathologic diagnosis necessary. Accordingly, a precise
neuroradiologic evaluation is of the utmost importance, because most lesions, if not
symptomatic, only require clinical and radiologic follow-up.
621
Choroid plexus tumors represent a well-defined subset of brain tumors that occur
mainly in young children. Surgical resection for papilloma is usually curative, although
careful surgical planning is required to minimize the potential risks. Although adjunctive
therapy for carcinoma includes chemotherapy or radiation, the long-term survival for
carcinoma remains poor.
CONTENTS
633
vii
FORTHCOMING ISSUES
January 2004
Endoscopy
Rick Abbott, MD, Guest Editor
April 2004
Traumatic Neurovascular Surgery
J. Paul Elliot, MD, Guest Editor
July 2004
Pain Treatment
Gary Heit, MD, Guest Editor
RECENT ISSUES
July 2003
Neuroaugmentation for
Chronic Pain
Jaimie M. Henderson, MD, Guest Editor
April 2003
Surgery for Psychiatric Disorders
Ali R. Rezai, MD, Steven A. Rasmussen, MD,
Benjamin D. Greenberg, MD, PhD
Guest Editors
January 2003
Pituitary Surgery
Martin H. Weiss, MD, and
William T. Couldwell, MD, PhD, Guest Editors
Preface
Intraventricular tumors
Neurosurgical oncology is an evolving discipline that continually benets from the translation
of scientic advances into clinical treatment paradigms. For the past 30 years at the University
of CaliforniaSan Francisco, basic scientists and
clinicians have been working together to rapidly
implement new discoveries for the benet of our
patients. Several examples of these collaborations
can be found here and at other premier neurosurgery departments around the world. A rened
understanding of the molecular pathways that
contribute to tumor development has yielded
new targets for chemotherapy, while our increasing experience with radiosurgery has broadened
treatment options for patients. The advent of surgical adjuncts such as functional mapping techniques, computerized frameless stereotaxy, and
endoscopy has signicantly decreased surgical
morbidity. In addition, surgical procedures are
now in place to facilitate local delivery of chemotherapeutic agents with unprecedented tumor
specicity.
Intraventricular tumors epitomize the challenges faced by neurosurgical oncologists in the
1042-3680/03/$ - see front matter 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S1042-3680(03)00059-7
Department of Neurological Surgery, University of California at San Francisco, 513 Parnassus, HSW 511,
San Francisco, CA 941430511, USA
b
Neuropathology Unit, Department of Pathology, University of California at San Francisco, 513 Parnassus,
HSW 408, San Francisco, CA 941430511, USA
* Corresponding author.
E-mail address: tihan@itsa.ucsf.edu (T. Tihan).
1042-3680/03/$ - see front matter 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S1042-3680(03)00060-3
470
papillary structures and do not exhibit architectural or cytologic atypia (Fig. 1A). Epithelial and
stromal cells contain many characteristics of the
normal choroid plexus, such as calcications and
xanthomatous change [9,10]. In some cases, there
is a striking nuclear monomorphism without
other aggressive features, such as mitoses or
vascular proliferation. Rarely, geographic necrosis without the pseudopalisading that is suggestive
of an infarct can be seen in an otherwise typical
CPP. Osseous or cartilaginous metaplasia and
acinar or tubular dierentiation are reported in
choroid plexus neoplasms [1113]. In addition,
a number of studies report a pigmented variant
that contains neuromelanin and lipofuscin [14].
CPPs with marked oncocytic transformation as
well as glial dierentiation are rare [12]. Transitional zones between the normal and neoplastic
choroid plexus can be found in CPPs and CPCs.
Carcinomas of the choroid plexus are tumors
that exhibit all the histologic hallmarks of
aggressiveness (see Fig. 1B). A typical CPC is
a neoplasm with increased architectural complexity demonstrating partially solid and partially
nonpapillary growth. Most tumors have marked
cytologic atypia, atypical mitotic gures, and
frank necrosis. Some high-grade tumors have
cytologic and architectural features that resemble
anaplastic oligodendrogliomas. Invasion into
neuropil is characteristic of CPCs, although some
CPPs can occasionally exhibit invasion into
surrounding parenchyma. Rare CPCs resemble
undierentiated carcinomas without any distinguishing features [15].
Immunohistochemical features
Cytokeratins and vimentin are expressed by
virtually all CPPs and most CPCs. Glial brillary
acidic protein (GFAP) can be found focally in
about 25% to 55% of CPPs and in 20% of CPCs
[16]. Most of the GFAP-positive cells are simul-
c
Fig. 1. (a) Choroid plexus papilloma: low magnication showing well-formed papillae composed of uniform small
epithelial type cells. Mitotic gures and necrosis are rare. (b) Choroid plexus carcinoma: a tumor with irregular
architecture, the presence of marked pleomorphic cells with a less prominent papillary pattern, and frequent mitoses and
necrosis. (c) Ependymoma: medium magnication showing uniform cells arranged in a perivascular fashion. (d )
Ependymoma: high magnication of an ependymal pseudorosette, an angiocentric arrangement of cells with brillary
processes perpendicular to the luminal axis. (e) Subependymoma: a paucicellular tumor showing a multinodular compact
architecture without mitotic gures. ( f ) Subependymal giant cell astrocytoma: a tumor composed of gemistocytic
astrocytes, scattered inammatory cells, and dystrophic calcications. (g) Central neurocytoma: a tumor typically
described as oligodendroglioma-like with clear cells (fried-egg cells) and a delicate vasculature (chicken-wire
vasculature). (h) Meningioma: a typical meningioma in the lateral ventricle. The tumor shows multiple whorl formation as
well as calcications known as psammoma bodies.
471
472
(see Fig. 1C, D). Some ependymomas are predominantly glial in appearance and may not have
distinct perivascular pseudorosettes, whereas
others may be predominantly epithelial. The latter
may present as a tumor with oval to round nuclei,
discrete cytoplasmic borders, frank papillary
structures, and well-formed brovascular cores.
Other tumors may show true ependymal rosettes distinguished by their well-dened lumina
and cells forming pseudoglandular structures.
Most ependymomas show a substantial number
of nuclear grooves that can be identied in
intraoperative smears and help with the rapid
interpretation of frozen sections [42]. This feature,
however, needs to be interpreted in the context of
other histologic ndings, because many other
tumors, such as meningiomas and other gliomas,
can exhibit nuclear grooves. The tumor nuclei are
uniform, round to oval, and often feature
a distinct nucleolus.
Clear cell change in ependymoma is a rare but
signicant nding [43]. Intraventricular ependymomas may exhibit focal or predominant clear
cell change. When clear cell change is predominant, the hematoxylin-eosin appearance of an
oligodendroglioma is recapitulated. It is likely
that many tumors previously reported as intraventricular oligodendroglioma are examples
of clear cell ependymoma [21]. Clear cell ependymomas are usually higher grade and exhibit
increased mitotic activity and vascular proliferation. The so-called tanycytic ependymoma is
remarkably similar to a pilocytic astrocytoma.
This highly brillary tumor has moderate cell
density, spindled cells, and a fascicular architecture. It has also been described as a piloid tumor
with ependymal nuclei [44]. The tanycytic
ependymoma often lacks nuclear pleomorphism
or aggressive features, such as mitoses or vascular
proliferation. Perivascular pseudorosettes are rudimentary and sometimes absent.
Ependymomas are commonly calcied and
rarely exhibit cartilaginous and osseous metaplasia. Rare ependymomas contain cytoplasmic
eosinophilic granules, clear vacuoles, lipid, or
melanin [45,46].
The current WHO classication denes grade
II ependymomas as tumors with mild cellular
pleomorphism, pseudorosettes, or true ependymal
rosettes. The tumors can have occasional mitotic
gures and necrosis without pseudopalisading.
Occasional foci of hypercellularity and increased
mitoses are allowed. Anaplastic, high-grade,
or grade III ependymomas have moderate to high
473
474
Epidemiology
475
476
of chromosome 22q in some tumors [66]. TSCassociated tumors also demonstrate loss of
heterozygosity in chromosomes 9 and 16, which
are known to harbor TSC genes [67]. One of two
suspected genes, TSC2, was found in chromosome
16 by positional cloning. The gene product from
TSC2 has been named tuberin. TSC1 was
discovered earlier in chromosome 9 but has not
yet been characterized. Genetic analysis on TSC
families reveals mutations in chromosome 9q34
(TSC1) and chromosome 16p13 (TSC2) as the
only common genetic anomalies [68].
The Eker rat, a naturally occurring animal
model of TSC, provides a powerful tool for
investigations of TSC. In this model, a conserved
linkage group on rat 10q corresponds to human
16p13.3 (TSC2 gene) [69]. Currently, it is believed
that the products of TSC1 and TSC2 genes
interact with each other in the cell.
Pathologic dierential diagnosis
SEGAs are fairly distinct intraventricular neoplasms that may be confused with gemistocytic
astrocytoma or high-grade glioma if the typical
pathologic and radiologic features are overlooked.
Small biopsies can also potentially be interpreted
as tanycytic ependymoma or subependymoma,
but this is less likely, because SEGAs are invariably more cellular, less brillary, and far more
gemistocytic.
Central neurocytoma
Epidemiology
The term central neurocytoma was rst used by
Hassoun et al [70] in 1982 to describe dierentiated intraventricular neuronal lesions observed in
2 cases. Central neurocytomas are rare neoplasms,
with 127 reported cases through 1993 [71].
Reported rates in series of pathologically conrmed primary CNS neoplasms range from 0.1%
to 0.5% [7274]. Central neurocytomas are
primarily tumors of young adults, with 45%
occurring in the third decade of life and almost
75% between the ages of 20 and 40 years [71].
Gender distribution is equal. Central neurocytomas arise predominantly from the septum
pellucidum or, less frequently, from the lateral ventricular wall. The anterior lateral ventricle is the
most frequent site (77%), followed by lateral and
third ventricle involvement (21%) [71]. Bilateral
lateral ventricular involvement is uncommon.
477
indicating neuronal dierentiation [78]. Synaptophysin antibody stains the brillar zones and, to
a lesser extent, the perinuclear cytoplasm of tumor
cells. Anti-Hu autoantibodies stain neurocyte
nuclei. Tumor cells are also positive for Leu-7 and
S-100 protein, whereas staining for GFAP is predominantly negative and vimentin is conned to the
nonneoplastic mesenchymal elements of blood
vessels [79]. Staining for myelin basic protein,
chromogranin, and neurolament is often negative.
Some studies have shown a small subpopulation of
GFAP-positive neoplastic cells, and glial dierentiation has been suggested in tissue culture. This
mixed phenotype of glial and neuronal marker
positivity in central neurocytoma can be interpreted as a glioneuronal neoplasm, with an overwhelmingly neurocytic component. In rare examples,
a tumor may have an increased Ki-67/MIB-1 index.
Such neoplasms are described as atypical neurocytomas and have a signicantly elevated incidence of local recurrence [80]. Even though no
clear cuto point exists between classic and atypical
neurocytomas, most authors suggest that tumors
with an MIB-1 index of greater than 2% be placed
in the atypical category. Nevertheless, some studies
show no dierence in MIB-1 labeling between
tumors with atypical features and typical central
neurocytomas [81]. Currently MIB-1 labeling is not
used to modify grading of central neurocytomas.
Ultrastructural features
Central neurocytoma is readily recognizable as
neuronal, with microtubules, terminations, clear
vesicles, and dense core granules [79,82,83]. Some
examples may display round cells with abundant
cell processes containing microtubules, cellular
junctions, and lysosome-like structures. Others
contain numerous synaptic vesicles, neuritic processes, and neurosecretory granules. In addition,
rare tumors contain ganglionic cells with welldeveloped processes.
Molecular and genetic features
Reported recurrent genetic changes in central
neurocytomas include alterations on chromosomes 2p, 10q, and 18q. The candidate genes in
these loci are currently unknown [84]. Other
studies have suggested gain of chromosome 7 as
a nonrandom genetic alteration in central neurocytomas [85]. Recent studies have demonstrated
that central neurocytomas are genetically distinct
from oligodendrogliomas and that chromosomes
1p and 19q probably do not play an important
478
Summary
Tumors that primarily or exclusively involve
the ventricular system constitute a rare and
heterogeneous group. Certain histologic tumor
types predominantly occur in children, whereas
others are more common in adults. Tumor
location provides additional clues to correct
diagnosis. When used in conjunction with clinical
and radiologic data, histopathologic features can
distinguish among this wide range of possibilities
to provide the correct diagnosis for optimal
patient management.
Acknowledgement
J.S. Waldron was supported in part by a grant
from the Khatib Research Foundation as a Khatib
Fellow 20022003.
[18]
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mutation in a pediatric ependymoma. Cancer
Genet Cytogenet 2002;138(2):10710.
[54] Scheinker I. Subependymoma: a newly recognized
tumor of subependymal derivation. J Neurosurg
1945;2:23240.
[55] Matsumura A, et al. Intracerebral subependymomas. Clinical and neuropathological analyses with
special reference to the possible existence of a less
benign variant. Acta Neurochir (Wien) 1989;96
(1 2):1525.
[56] Scheithauer BW. Symptomatic subependymoma.
Report of 21 cases with review of the literature.
J Neurosurg 1978;49(5):68996.
[57] Prayson RA, Suh JH. Subependymomas: clinicopathologic study of 14 tumors, including comparative MIB-1 immunohistochemical analysis with
other ependymal neoplasms. Arch Pathol Lab
Med 1999;123(4):3069.
[58] Moss TH. Observations on the nature of subependymoma: an electron microscopic study.
Neuropathol Appl Neurobiol 1984;10(1):6375.
[59] Dal Cin P, et al. Cytogenetic investigation in
subependymoma. Cancer Genet Cytogenet 1999;
108(1):84.
[60] Wiederholt WC, Gomez MR, Kurland LT. Incidence and prevalence of tuberous sclerosis in
Rochester, Minnesota, 1950 through 1982. Neurology 1985;35(4):6003.
[61] Shepherd CW, et al. Subependymal giant cell
astrocytoma: a clinical, pathological, and ow
cytometric study. Neurosurgery 1991;28(6):8648.
[62] Altermatt HJ, Scheithauer BW. Cytomorphology
of subependymal giant cell astrocytoma. Acta
Cytol 1992;36(2):1715.
[63] Hirose T, et al. Tuber and subependymal giant cell
astrocytoma associated with tuberous sclerosis: an
immunohistochemical, ultrastructural, and immunoelectron and microscopic study. Acta Neuropathol (Berl) 1995;90(4):38799.
[64] Lopes MB, et al. Immunohistochemical characterization of subependymal giant cell astrocytomas.
Acta Neuropathol (Berl) 1996;91(4):36875.
[65] Gyure KA, Prayson RA. Subependymal giant cell
astrocytoma: a clinicopathologic study with
HMB45 and MIB-1 immunohistochemical analysis. Mod Pathol 1997;10(4):3137.
[66] Debiec-Rychter M, et al. Cytogenetic changes in
two cases of subependymal giant-cell astrocytoma. Cancer Genet Cytogenet 1999;109(1):
2933.
[67] Sampson JR, Harris PC. The molecular genetics
of tuberous sclerosis. Hum Mol Genet 1994;3:
147780.
[68] Janssen B, et al. Rened localization of TSC1 by
combined analysis of 9q34 and 16p13 data in 14
tuberous sclerosis families. Hum Genet 1994;
94(4):43740.
481
482
Intraventricular neurocytomas
Janet Lee, MS, Susan M. Chang, MD, Michael W. McDermott, MD,
Andrew T. Parsa, MD, PhD*
Department of Neurological Surgery, University of California at San Francisco, 505 Parnassus Avenue,
M-779, San Francisco, CA 94143, USA
Background
Epidemiology and clinical presentation
A review of 385 reported neurocytoma cases
[196] shows that, in general, central neurocytomas (CNCs) are well-dierentiated intraventricular tumors that aect young adult men and
women equally. Most commonly, CNCs occur in
the anterior portion of the lateral ventricle around
the foramen of Monro and can attach to either the
septum pellucidum or the lateral wall of the
ventricle [19,58,71,97]. Whereas 75% of cases
occur in patients between the ages of 20 and
40 years [15,28,98], CNC occurring in patients
18 years of age or younger [3,5,14,19,32,36,42,
44,58,68,69,71,74,76,78,88,94] and in patients 50
years of age or older [3,4,7,19,40,44,45,53,69,
76,78,93,99] have been reported. The term
extraventricular neurocytoma is used to describe
histologically similar tumors not found in intraventricular locations [43]. Extraventricular
locations include the occipital lobe [8,22,54,72],
parietal lobe [3,22,54], frontal lobe [8,22,24,58,96],
temporal lobe [8,9,22,58], thalamus [8,72,100],
hypothalamus [8,22,69], cerebellum [7,17], pons
[77], spinal cord [3,12,18,44,47,79,82,83], cauda
equina [81], retina [52], and pelvis [101] as well as
mature cystic teratoma of the ovary [102]. Cases
1042-3680/03/$ - see front matter 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S1042-3680(03)00064-0
484
Table 1
Signs and symptoms
Signs and Symptoms
No.
HA
N/V without HA
Dizziness
Visual disturbance
Altered mental status
Seizure
Motor disturbance
Sensory disturbance
Papilledema
178/202
2/202
4/202
51/202
22/202
9/202
40/202
14/202
46/51
88.1
1.0
2.0
25.2
10.9
4.5
19.8
6.9
90.2
Neuroimaging
CT/MRI/angiography
Appropriate diagnostic imaging studies for
patients with CNC may include CT, MRI, or
angiography. Relative to the brain parenchyma,
CNC appears as a well-circumscribed, isodense,
hyperdense, or mixed isodense/hyperdense mass
with slight to moderate contrast enhancement on
CT examination. Noncommunicating (obstructive) hydrocephalus is often present. Calcications
and cyst-like areas may also be seen on CT
images. Compared with the surrounding white
matter, T1-weighted and proton-weighted images
typically appear isodense. T2-weighted images
appear heterogeneous with areas of calcication
and cysts appearing hyperintense and the tumor
appearing isointense to hyperintense. Variable
enhancement with gadolinium is common, reecting the heterogeneous vascularity of CNC (Fig. 1).
For tumor localization and visualization of the
485
Fig. 1. (A) Axial CT image with contrast. (B) Contrast-enhanced coronal T1-weighted MRI. (C) Axial T1-weighted
MRI. (D) Sagittal T1-weighted MRI.
486
Fig. 1 (continued )
487
Treatment
Overview
Fig. 3. (A) Central neurocytoma showing immunoreactivity for synaptophysin (synaptophysin immunohistochemistry, original magnication 20). (B) Electron
microscopy of central neurocytoma. The tumor cells
have round nuclei and clear cytoplasm. The cytoplasm
contains microtubules, dense core vesicles, and synapses
(*) (original magnication 10,500). (From Hara M,
Aoyagi M, Yamamoto M, Maehara T, Takada Y, Nojiri
T, et al. Rapid shrinkage of remnant central neurocytoma after gamma knife radiosurgery: a case report.
J Neurooncol 2003;62(3):26973; with permission.)
vascular pseudorosettes, neuropil islands, multinucleate cells, or ganglion cells. Mitotic activity
can be as high as 30 mitoses per high-power eld,
and evidence of necrosis may range from focal to
extensive. Although the correlation between
histologic atypia and proliferation potential in
atypical CNC was poor [110], vascular proliferation showed a signicant correlation with the
MIB-1 labeling index (LI) (P = 0.0006) [77]. It is
unclear how the histology of atypical CNC relates
to biologic behavior. Although elevated prolifer-
488
Table 2
Larger case series
MIBY
labeling
n index
Location
Primary
treatment
Recurrence
Average
months to
recurrence
(range)
Radiation
Timing
of RT
Average
FU in
months
(range)
Local
control
Survival
rate
Outcome
Reference
Not
reported
3 LLV, 1
LLV/3rd
4 CTR
4/4
17.25
(925)
GKS, 1620
Gy to tumor margin
4 salvage
20.25 p
0% p initial
GKS, 54.5 surgery,
p CTR
100% p GKS
[2]
100.0% Returned to work with
full fxn, 3/4 neurologically
nl, 1/4 on dilatin for
postoperative seizure
3.6375
9 intraventricular
6 ITR, 3
CTR, 3 RT
2/9
618
55 Gy (2 adjuvant, 1
salvage for
asymptomatic
progression)
2 adjuvant,
1 salvage
45.33
(689)
83.30%
91.7%
Not
reported
3 LLV,
1 BLV
53
GKS, 913 Gy (3
adjuvant, 1 salvage)
3 adjuvant,
1 salvage
44
(1299)
100%
100.0% 3 asymptomatic, 1
neurologically intact
18 Not
reported
2 3rd, 3 3rd/
LV, 2 CC, 1
CC/SP, 8
FOM/LV,
2 SS
8/18
4 biopsy/
VPS/RT, 7
CTR, 7 ITR,
1 chemo,
7RT
NS
NS
7 adjuvant
46.4
(684)
44.40%
83.0%
10 1.9
(0.15.6)
Not
reported
8 ITR/RT, 1 1/10
ITR, 1 ITR/
RT then
CTR
At least
12
90
(23160)
100%
2 LLV, 1
midline, 3
LLV/3rd
NS
NS
NS
15
(0.536)
NS
Not
reported
NS
NS
[3]
[11]
5 RLV, 1
LLV, 2
BLV, 2
BLV/3rd, 1
RLV/3rd, 1
LLV/3rd, 3
NS
7 CTR, 8
ITR 7 RT
2/15
821
66.1
(18168)
75% surgery
only, 100%
surgery/RT
[39,
40]
Not
reported
2 BLV, 3
LLV, 1
LLV/3rd, 1
RLV
3 CTR, 4
ITR, 2 RT
NA
60 Gy
Adjuvant
52.7
NS
87.5%
[41]
Not
reported
7 LV, 1 3rd
2 stereotactic 1/8
Bx/RT, 5
stereotactic
Bx/RT/
chemo
15
50 Gy whole
brain (180 cGY
56 weeks)
Primary
78 (15
108)
88%
86.0%
[42]
1 lost to follow-up; 5
asymptomatic, employed,
KPS >90; 1 shunt
surgery at 9 years after
initial RT, employed,
KPS >90; 1 died as a
result of shunt
dysfunction at 5 years
after initial RT
Not
reported
3 RLV, 2
LLV/3rd
1 ITR, 2
ITRNPS/
RT, 2
ITRNPS/
chemo/RT
NA
1, RT, 52.5 Gy to
tumor, 3 RT, 54.0
Gy to tumor/30
Gy to axis
Adjuvant
23.5 (11
78)
NS
2 Bx/obs, 8 n
CTR, 5 ITR,
5 RT
NA
NS
NS
72.8 (13
255)
71.40%
93.3%
[45]
1 lost to follow-up, 10
alive and well, 3
asymptomatic recurrence,
1 died at 1 month
NA
NA
NA
NS (up to NS
50)
80.0%
[46]
1 recovered with slight
hemiparesis at 5 y, 1 lost
to FU p 3 months, 1 died
after surgery, 2 totally
recovered after surgery
15 1.9 (0.16) 15
ventricular
[44]
Not
reported
2 LLV/3rd, 1 3 CTR, 2
ITR
LLV, 1
BLV/3rd, 1
BLV
Not
reported
2 RLV, 2
LLV/3rd
1 Bx/obs, 2 n
CTR, 1 ITR,
1 RT
NA
50 Gy
Adjuvant
50.25 (4
135)
80%
100.0% 2 no recurrence, 2 no
regrowth
[53]
Not
reported
6 LLV, 1
BLV/3rd, 1
BLV
6 ITR, 2
ITR, 3 RT
NS
NS
3 adjuvant
120.5
(67.2
181.2)
NS
[58]
NS
489
15 9
proliferating cell
nuclear
antigen
<1%
490
Table 2 (continued)
Radiation
Timing
of RT
NA
NS
3 adjuvant
2 BLV, 2
RLV, 3 LLV
2 GTR, 4
STR, 1
BxNPS/RT,
2 RT
NA
NS
32 Not
reported
10 LLV, 10
RLV, 11 BLV,
1 hypothalamus
NS
5 GTR,
5 GTR/RT,
14 STR,
8 STR/RT
NS
1 RLV, 1
LLV, 1 SS
3 ITR,
2 RT
Location
4* Not
reported
7
6 <1%
Not
reported
Primary
treatment
1/3
Average
FU in
months
(range)
Local
control
Survival
rate
Outcome
Reference
[60]
3 adjuvant
10122
100%
85.7%
5 alive, asymptomatic; 1
death; 1 alive with
considerable neurologic
decits
[99]
11 RT, 48.661.2 Gy
to tumor bed, 2 RT,
3036 Gy to
craniospinal axis for
adjuvant RT
13 adjuvant,
3 salvage
56.4
(28184)
79%
81.0%
NS
[70]
NS
1 salvage
at 9 mo
30, 34 ys,
10
66.70%
66.7%
[71]
NA
4060 Gy over 6
weeks
Adjuvant
32 (672)
100%
75.0%
[78]
29 alive, 4 NS, 1 dead
with cerebral edema after
surgery, 1 dead with
massive IVH 6 weeks after
surgery, dead with brain
stem infarct 1 month after
recurrence noted at 4
months
20 0.75
(0.13)
14 CTR, 6
n (4 lost
10 RLV, 4
LLV, 4 LLV, 1 ITR, 15 RT to FU)
LLV/3rd, 1
3rd
36 3.4
(0.121)
36 LV
8/36
34 CTR,
1 stereotactic
Bx/RTCTR
for residual,
1 biopsy/RT
27.75
(473)
46.9 (0.5
204)
NS
89.7%
Not
reported
1 LLV, 1 LV/
3rd, 1 3rd/
hypothalamus, 1
3rd/thalamus
1/4
2 biopsy/
GKS, 1
CTR, 1 ITR
2/GKS
48
GKS, 1420 Gy to
tumor margin, 2840
Gy maximum
2 primary,
1 salvage,
1 adjuvant
45.75
100%
p GKS
Not
reported
18
1 salvage,
3 adjuvant
1/5
[89]
Recurrence
Average
months to
recurrence
(range)
MIBY
labeling
n index
10 Not
reported
2 LV, 4 SP, 1
CC, 4 FOM
NS
1/10
24
NS
6 LV, 1 LV/
3rd/4th
6 CTR, 1
ITR, 1 RT
3/7
36, 38, 72
Not
reported
NS
NS
NS
NS
NS
[98]
61.8 (5
143)
50%
[96]
100.0% 6 returned to work;
1 residual hemiparesis;
3 shunt at 2 mo, 1 mo and
1 y; 1 asymptomatic
recurrence, obs
491
492
radiation side eects; however, long-term followup data are lacking [2,6,11,36,48,65,88]. Chemotherapy has also been used in a limited number of
cases [7,14,18,42,44,71,88]. Outcome measures
used to assess ecacy of therapeutic interventions
include local control, time to progression or
recurrence, survival, and functional performance.
Although most patients with intraventricular
tumors present with symptoms of obstructive
hydrocephalus, the advent of CT and MRI has
increased detection of asymptomatic tumors [15,
22,32,53,60,87,91]. Management of patients with
asymptomatic CNC is largely unexplored in the
published literature. Indications for treatment
usually include signs and symptoms caused by
the tumor. Consequently, a practical approach to
patients harboring an intraventricular tumor with
characteristic features of a CNC may be to delay
intervention until symptoms occur [58]. By extension, judicious observation may be appropriate
for patients with asymptomatic recurrence or
progression.
Given that initial management in many CNC
patients was based on an incorrect diagnosis [2,
3,14,40,42,46,60,65,68,89,90,93], there is no clear
management or treatment paradigm for primary,
recurrent, or progressive CNC in the literature
[36]. In addition, long-term follow-up for patients
is not standardized. The following section discusses treatment management for symptomatic
CNC, including prospective observation, surgery,
RT, radiosurgery, and chemotherapy for primary
and recurrent CNC.
Observation
Because the indications for surgery usually
include signs and symptoms caused by the tumor
[58], one approach to asymptomatic tumor
management is observation with close follow-up.
One of the rst reported cases of CNC was
initially treated with a ventriculoperitoneal shunt
(VPS), followed by observation for an unspecied
period [27]. The patient later underwent biopsy
and GTR after symptomatic progression. Mackenzie [45] has reported two cases of CNC that
were observed after biopsy, and both patients are
alive and well with no progression at 35 and 255
months of follow-up, respectively. The MIB-1 LI
for these biopsies showed MIB-1 LIs of 0.1% and
1.8%, respectively. Giangaspero and colleagues
[22] have reported one case of a parietal neurocytoma discovered incidentally after biopsy. This
patient had stable disease at the 6-month follow-
up examination but showed progression at the 26month follow-up examination. The LI of this
tumor was 1% to 1.5%. Mineura and colleagues
[53] also have reported one case of CNC
discovered incidentally after biopsy. No progression was noted at the 51-month follow-up
examination. Yasargil and colleagues [96] have
reported two cases of asymptomatic recurrent
CNC 3 and 6 years after GTR. The patients were
observed and were alive and well at last follow-up
at 58 and 92 months, respectively.
Takao and colleagues [82] have reported a case
of CNC that was observed after biopsy because
of pregnancy. STR was performed 11 months
later because of symptomatic progression. As the
result of postoperative MRI revealing residual
tumor with spinal cord dissemination, the patient
underwent RT (66 Gy, cone technique, extended
eld at 40 Gy, reduced eld at 20 Gy, with use of
limited eld size for a nal boost of 20 Gy to the
tumor bed and 46 Gy to the spinal cord, 2 Gy/d,
4 fractions per week), resulting in a decrease in
tumor size and stable disease at the 3-month
follow-up examination. Agranovich and colleagues [1] have reported a case of CNC that
presented as IVH on imaging and was followed
with serial CT scans because the patient declined
surgery. After subsequent biopsy and MRI 3
years later because of symptomatic progression,
the patient received RT (50 Gy in 25 fractions
over 5 weeks, isocentric technique [6.5 cm 5
cm, 7 5 eld size] with a 6-MV energy linear
accelerator [LINAC]) and was asymptomatic
with stable tumor size at the 3-year follow-up
examination.
Because of the benign clinical course of CNC,
prospectively observing patients after biopsy until
symptomatic progression of the tumor may be
a reasonable approach. The MIB-1 LI may help
to stratify patients into high-risk and low-risk
groups. Observation may also play a role in tumor
management after asymptomatic progression or
recurrence; however, this approach should be used
with caution, because recurrent tumors demonstrate proliferation that may indicate a more
clinically aggressive tumor (Table 3).
Acute management
Because many patients present with symptoms of increased ICP, acute management may
necessitate the use of temporizing measures
before a more denitive treatment is administered. Treatment of acute noncommunicating
493
Months to
recurrence
or progression
Treatment for
recurrence
or progression
36
Not stated
Biopsy/observation
Biopsy/observation
Biopsy/observation
Biopsy/observation
us a resulted
pregnancy
GRT
n
n
n
y
11
36
Observation
asymptomatic
58 p GRT
GRT
72
Observation
asymptomatic
92 p GTR
Primary
treatment
Observation with CT
patient refused
surgery
VPS/observation
Follow-up
in months
Outcome
Reference
Stereotactic biopsy
and GTR
36 p GTR
Asymptomatic
[1]
Stereotactic
biopsy and RT
STR/RT
Not stated
Alive, no
recurrence
Alive and well
Alive and well
No regrowth
Alive and well
[27]
35 p biopsy
255 p biopsy
51 p biopsy
3 p STR/RT
Returned to work,
asymptomatic
recurrence
Returned to work,
asymptomatic
recurrence
[45]
[53]
[82]
[96]
Abbreviations: GTR, gross total resection; n, no; RT, radiation therapy; STR, subtotal resection; VPS,
ventriculoperitoneal shunt; y, yes; p, post.
determining a safe plan for resection is identication of the ependymal surface of the oor of the
ventricle anterior and posterior to the tumor. The
choroid plexus and ependymal veins can then be
used as guides for dissection along the inferior
aspect of the tumor.
Surgical resection: gross total resection versus
subtotal resection
Because of the benign nature of the disease,
GTR and STR have resulted in a stable long-term
outcome. After GTR, disease-free survival has
been reported up to 12.5 years [41]. Similarly,
after STR, stable disease has been reported up to
18.5 years [45]. Recurrences have been reported
after STR and GTR, however [18,39,40,72,96].
There is an inherent bias in the STR group,
because those tumors may be more extensive.
In a retrospective review of 32 cases of patients
who received multimodality therapy, Schild and
colleagues [70] compared GTR RT and
STR RT and found a 5-year local control rate
of 70% for patients after STR RT compared
with 100% for patients after GTR RT (log rank
rest of Kaplan-Meier product limit method projection, P = 0.08). Adjuvant conventional external beam RT in initial treatment ranged from 48.6
to 61.2 Gy in 180- to 200-cGy fractions delivered
to the tumor bed in 11 patients. Two patients
received adjuvant craniospinal and whole-brain
494
Table 4
Surgical approaches
Surgical approach
No. patients
References
Transcallosal
Transcortical
Transventricular
Combined
Biopsy
Not specied
31
30
1
17
28
194
[5, 11, 14, 15, 26, 35, 39, 63, 74, 76, 85, 88, 89, 91, 96]
[1, 7, 14, 15, 32, 35, 36, 40, 43, 46, 53, 62, 68, 71, 75, 82, 88, 89]
[57]
[2, 7, 31, 40, 53, 60, 63]
[7, 14, 19, 28, 30, 42, 45, 53, 78, 82, 88, 99, 105, 108]
[3, 6, 10, 11, 19, 20, 23, 27, 30, 33, 41, 44, 45, 48, 58, 60, 65, 70, 78, 85,
87, 9395, 99]
paresis [11,15,16,32,46,68] meningitis [27], hemorrhage [43,78], and death [41,43,46,70,78], after
surgery have been noted, but most patients have
an uncomplicated postoperative course (Table 5).
Surgery for recurrence/progression
CNC can recur or progress, and some authors
have reported their experience with repeat surgery
[2,10,78,96]. Over the years, the small but demonstrated risk of morbidity associated with surgery
has provided the impetus to pursue alternative
treatments for recurrence. Accordingly, surgery
for recurrence or progression has decreased in
frequency over the years as other therapeutic
options have become available [40,62,71].
Stereotactic biopsy
In cases where initial treatment does not
include surgical resection, a tissue biopsy must
be obtained to establish a denitive diagnosis.
Although histologic atypia has not been shown to
correlate with clinical outcome [39,40,99], proliferation potential has been correlated to prognosis. Evaluation of proliferation potential using
the MIB-1 LI can be used to guide further
therapy. MIB-1 is a monoclonal antibody that is
more durable than the original Ki-67 antibody
and is used as a nuclear proliferation marker.
Nuclei positive for MIB-1 are easier to count than
nuclei positive for proliferating cell nuclear
antigen (PCNA), and the results are comparable
to those of bromodeoxyuridine (BUDR) analysis
(Fig. 5) [51].
In a retrospective case series evaluating proliferation potential using the MIB-1 LI, Soylemezoglu and colleagues [78] reported that tumors
with an MIB-1 LI greater than 2% (39% of cases)
have a signicantly greater chance of relapse
(63%) over an observation period of 150 months
compared with cases with a lower MIB-1 LI (22%
relapse) (v2 test of Kaplan-Meier analysis using
one degree of freedom, P = 0.08). An MIB-1 LI
495
Table 5
Postoperative complications within the rst 3 months
No.
After gross total resection
(n = 127)
None
Death
Transient hemiparesis
Persistent hemiparesis
Shunt within 1 month
Meningitis
Cognitive dysfunction
Comatose <3 weeks
Decreased vision
108
8
1
6
2
1
5
1
2
85.0
6.3
0.8
4.7
1.6
0.8
3.9
0.8
1.6
85
3
2
4
3
4
1
1
1
84.8
3.0
2.0
4.0
3.0
4.0
1.0
1.0
1.0
After biopsy
(n = 28)
None
28
100
496
the experience is limited and the reported followup data are brief. If the tumor is small and
patients are closely observed for tumor progression, focal RT after biopsy may be a possible
treatment option in centers that lack stereotactic
radiosurgery.
Role of radiotherapy after gross total resection.
Rades and Fehlauer [112] have reported that the
3-year local control rate showed no statistical
dierence between GTR (95%) and GTR/RT
(96%) (RT dose not specied). Similarly, local
control at 5 years showed no statistical dierence
between these two groups (85% for GTR and
89% for GTR/RT). Data from Schild and
colleagues [70] support these ndings and demonstrate that GTR resulted in 5-year local control
and survival rates of 100% and 80%, respectively.
Median time to progression was 36 months after
GTR and 39 months after GTR/RT with 5-year
survival rates of 99% for GTR and 95% for
GTR/RT (dose not specied by patient). Although the number of patients in the GTR/RT
group was small (n = 35), these data suggest that
RT should not be used as an adjuvant in patients
after GTR, because there is no proven benet.
Role of radiotherapy after subtotal resection for
treatment of residual tumor. Schild and colleagues
[70] have reported that among patients who
underwent STR, the 5-year local control rate
was 100% for patients who received postoperative
RT and 50% for patients who did not (log rank
rest of Kaplan-Meier product limit method projection, P = 0.02). The 5-year survival rate after
STR also showed a trend for longer survival in
patients who received postoperative RT (88%)
compared with patients who did not (71%) (logrank rest of Kaplan-Meier product limit method
projection, P = 0.3). Brown and colleagues [9]
also found that crude local control rates for STR
versus STR/RT (48.661.2 Gy in 180200 cGy
fractions or 59-Gy median dose) were 62% and
100% (two-tailed test, P = 0.0008), respectively.
In a larger study, Rades and Fehlauer [112] have
reported a 3-year local control rate of 55%
compared with 89% among patients who underwent STR and STR/RT, respectively. At 5
years, local control rates were 46% for STR and
83% for STR/RT (log rank rest of Kaplan-Meier
projection, P<0.001). Median time to progression
was 20 months after STR and 34 months after
STR/RT; however, 5-year survival rates were 86%
for STR and 90% for STR/RT and showed no
497
498
Table 6
Stereotactic radiosurgery and outcome
Recurrence
in months
Radiation
GTR
GKS, 16 Gy
GTR
25
GKS, 16 Gy
GTR
21
GKS 20, Gy
GTR
14
GKS, 16 Gy
GTR 2
6072
GKS, 12.5 Gy
Timing of
radiation
Salvage at 9
months
Reoperation with
GKS salvage
at 25 months
Reoperation with
GKS salvage
at 21 months
Salvage at 14
months
Salvage at 5 to 6
years
Initial size
in cc cubic
centimeters
Follow-up
MRI
Follow-up
in months
Decrease in
tumor size
Decrease in
tumor size
14 p GKS, 24
p GTR
28 p GKS, 83
p GTR
1.7
Decrease in
tumor size
12 p GKS, 84
p GTR
7.9
Decrease in
tumor size
58% decrease in
tumor size after
GKS
27 p GKS, 27
p GTR
12 p GKS
40% decrease
in tumor size
after GKS
61% decrease
in tumor size
after GKS
48% decrease
in tumor size
72% decrease in
post op tumor
81% decrease
in tumor size
77% decrease
in tumor size
60 p GKS
6.2
12.3
0.6
GTR
GKS, 13 Gy
5.2
GTR 2
GKS, 9.616
Gy
5.9
STR/GKS
GKS, 9 Gy
STR/GKS
GKS, 13 Gy
STR/GKS
GKS, 10 Gy
GTR
53
GKS, 10 Gy
Adjuvant 8
months later
Adjuvant 18
months later
Adjuvant
Salvage at 53
months
6.5
13
29
10.5
Outcome
Reference
[116]
Died us a result of
cardiac failure caused
by pericarditis 1 year
p GKS, 2/3 returned
to work, 3/3 no new
neurologic problems,
1/3 had persistent
abducens palsy and
visual impairment
[6]
99 p GKS
Neurologically intact
[11]
23 p GKS
Asymptomatic
42 p GKS
Asymptomatic
12 p GKS
Asymptomatic
24 p GKS
Primary
treatment
499
GKS, 20 Gy
Adjuvant
5.7
STR/GKS
GKS, dose
not stated
Adjuvant
Not stated
STR
Biopsy/GKS
Not
GKS, 15 Gy
GKS, 15 Gy
Salvage
Primary
Not stated
4.2
STR 2/
GKS
Biopsy/GKS
GKS, 14 Gy
Adjuvant
7.9
GKS, 18 Gy
Adjuvant
Not stated
GTR/
radiosurgery
72
Radiosurgery,
50 Gy
Adjuvant
Not stated
GTR
Radiosurgery
Salvage at 8
months
1.2
GTR
36
Salvage at 3 years
2.7
STR
12
Radiosurgery
18 Gy
LINAC, 17.5
Gy
Salvage after 6
months of
observation
Not stated
STR/RT
144,
36, 36
30 Gy at
presentation/
50 Gy at
recurrence 2/
LINAC at
recurrence 3
Adjuvant and
salvage
Not stated
2, 4, 6, 8, 10, and 12
months after GKS,
tumor shrank at
2 months
Shrinkage of tumor
at 21 months
12
Neurologically intact
[26]
21 p GKS
[35]
Stable disease
Signicant decrease
in tumor size at 40
months
Complete regression
at 18 months
Minimal decrease in
tumor size at 25
months
Complete remission
p initial Tx, 6
years later
recurrence
6 months p
radiosurgery
13
50 p GKS
No progression,
shrinkage of tumor,
KPS full
Stable disease
No new neurologic
problems
Signicantly smaller
tumor p
radiosurgery
Decrease in tumor
size at 6 months p
LINAC,
disappeared by 36
months p LINAC
Not stated
53 p GKS
25 p GKS
87 mo p
radiosurgery
No new clinical
symptoms
Normal neurologic
examination
[70]
[89]
[8]
Stable disease no
progression (received
chemo 72 months p
radiosurgery)
KPS 100
[7]
Neurologically intact
[65]
51 p LINAC
Neurologically intact
[36]
60 p LINAC,
240 p initial
ITR
[30]
10 p
radiosurgery,
18 p GTR
34 p
radiosurgery
[40]
Abbreviations: chemo, chemotherapy; fxn, function; GTR, gross total resection; KPS, Karnofsky performance scale; LINAC, linear accelerator; nl, normal; p, post; RT,
radiation therapy; STR, subtotal resection; Tx, therapy.
500
STR/GKS
501
502
involves several challenges: (1) inherent or acquired mechanisms of resistance, (2) eective drug
delivery, and (3) altered drug metabolism caused
by interactions with anticonvulsants and steroids.
Chemotherapeutic regimens often involve combining drugs with dierent mechanisms of action
and nonoverlapping toxicities. Some chemotherapeutic agents may also cause radiosensitization.
In general, histopathologic grade plays a key prog-
Table 7
Chemotherapeutic regimens
Primary
treatment
Chemo
Recurrence in
months
Follow-up
in months
after chemo
Outcome
72
15
60
18
38
36
22
96
Stereotactic Bx/RT/
chemo
Stable disease, no
progression
Complete remission for 36
months
Full-time employment, no
progression
60
Stereotactic Bx/RT/
chemo
Stereotactic Bx/RT/
chemo
Adjuvant loumustine,
nine dosesb
Adjuvant loumustine,
seven dosesa
15
15
108
Stereotactic Bx/RT/
chemo
Stereotactic Bx/RT/
chemo
Stereotactic Bx/RT/
chemo
STR/VPS/chemo/RT
STR/VPS/chemo/RT
2 GTR/RT/chemo
Adjuvant loumustine,
eight dosesb
Adjuvant loumustine,
nine dosesa
Adjuvant loumustine,
seven dosesa
Adjuvant cytoxan, cisplatinb
Adjuvant cytoxan, cisplatinb
Adjucant cisplatin plus
lomustineb
Adjuvant lomustine aloneb
Adjuvant lomustine plus
carmustineb
Adjuvant vincristine,
lomustine, prednisoneb
90
114
96
GTR/RT, stereotactic
Stereotactic
biopsy/RT
GTR
Shunt, cells for
cytology, STR/
chemo
2 STR/RT/chemo
Not
stated
14
11
Not
stated
Abbreviations: Bx, biopsy; chemo, chemotherapy; GTR, graps total resection; KPS, Kurnofsky performance scale,
RT, radiation therapy; STR, subtotal resection; VPS, ventroperitoneal shunt.
a
Schedule not specied.
b
Dose and schedule not specied.
503
504
Table 8
Outcome measures
Outcome measure
No. patients
References
Survival
181
Shunt failure
Local control (recurrence,
progression, stable disease)
Change in neurologic examination
Functional descriptiona
5
153
[17, 10, 11, 14, 15, 18, 19, 20, 23, 26, 27, 31, 35, 36, 4046, 53, 56
58, 60, 6265, 68, 70, 74, 78, 82, 85, 8789, 9496, 99, 104, 108]
[27, 42, 70]
[24, 6, 7, 10, 11, 14, 15, 18, 19, 20, 26, 27, 30, 31, 35, 36, 40, 42, 45,
53, 57, 58, 60, 62, 6365, 68, 70, 78, 82, 85, 8789, 9496, 99, 104]
[1, 2, 5, 6, 11, 15, 23, 25, 36, 46, 57, 65, 68, 88, 96, 99]
[1, 2, 5, 6, 10, 14, 15, 23, 35, 4042, 44, 45, 56, 58, 60, 63, 68, 96, 99]
26
98
505
506
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[54] Moller-Hartmann W, Krings T, Brunn A, Korinth
M, Thron A. Proton magnetic resonance spectros-
[55]
[56]
[57]
[58]
[59]
[60]
[61]
[62]
[63]
[64]
[65]
[66]
[67]
[68]
[69]
[70]
[71]
507
508
* Corresponding author.
E-mail address: rca24@columbia.edu
(R.C.E. Anderson).
1042-3680/03/$ - see front matter 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S1042-3680(03)00054-8
510
511
Fig. 1. Anatomic locations and tumor pathology by age group. PNET, primitive neuroectodermal tumor; CPP, choroid
plexus papilloma; GBM, glioblastoma multiforme; F, foramen; SGCA, subependymal giant cell astrocytoma. (From
Jelinek J, Smirniotopoulos JG, Parisi JE, Kanzer M. Lateral ventricular neoplasms of the brain: dierential diagnosis
based on clinical, CT, and MR ndings [Fig. 1]. AJNR Am J Neuroradiol 1990;11(3):56774; with permission.)
512
Fig. 2. Structures in the walls of the lateral ventricles. The central diagram shows the level of the cross-sections through
the frontal horn (A), body (B), atrium (C), and temporal horn (D). (A) Frontal horn. The genu of the corpus callosum is
in the roof, the caudate nucleus is in the lateral wall, the rostrum of the corpus callosum is in the oor, and the septum
pellucidum is in the medial wall. (B) Body of the lateral ventricle. The body of the corpus callosum is in the roof, the
caudate nucleus is in the lateral wall, the thalamus is in the oor, and the septum pellucidum and fornix are in the medial
wall. The choroidal ssure, the site of the attachment of the choroid plexus in the lateral ventricle, is situated between the
fornix and the thalamus. (C) Atrium. The lateral wall and roof are formed by the tapetum of the corpus callosum, and
the oor is formed by the collateral trigone, which overlies the collateral sulcus. The inferior part of the medial wall is
formed by the calcar avis, the prominence that overlies the deep end of the calcarine sulcus, and the superior part of the
medial wall is formed by the bulb of the corpus callosum, which overlies the forceps major. (D) Temporal horn.
The medial part of the oor of the temporal horn is formed by the prominence overlying the hippocampal formation,
and the lateral part of the oor is formed by the prominence called the collateral eminence, which overlies the deep end of
the collateral sulcus. The roof is formed by the caudate nucleus and the tapetum of the corpus callosum, the lateral wall
is formed by the tapetum of the corpus callosum, and the medial wall of the temporal horn is little more than the cleft
between the mbria of the fornix and the inferolateral aspect of the thalamus. Call., callosum; Coll., collateral; Corp.,
corpus; Hippo., hippocampus; Nucl., nucleus; Pell., pellucidum; Sept., septum; Sulc., sulcus; Trig., trigone. (From
Rhoton AL, Jr. The lateral and third ventricles [Fig. 5.4]. Neurosurgery 2002;51(4 Suppl 1):S20771; with permission.)
513
Fig. 3. Frontal transcortical approach into the right lateral ventricle. This opening exposes the caudate nucleus, fornix,
foramen of Monro, thalamus, thalamostriate vein, and choroid plexus. The inset on the lower right shows the site of the
cortical incision. (From Rhoton AL, Jr. The lateral and third ventricles [Fig. 5.25]. Neurosurgery 2002;51(4 Suppl 1):
S20771; with permission.)
ssure is arguably the single most valuable intraventricular landmark for the neurosurgeon.
The choroid plexus runs parallel and lateral to
the fornix and is attached by the taenia at the
choroidal ssure, which lies between the fornix
and the thalamus. The choroid plexus is thus
found in the medial aspect of the body, atrium,
514
Operative intervention
Preoperative considerations
MRI is the preferred modality for preoperative
evaluation because it provides the best threedimensional images of the tumor and the surrounding neurovascular structures. It can also give
clues regarding the vascularity of the tumor. CT
provides reasonable detail when MRI is contraindicated, and areas of calcication or hemorrhage
are more easily seen with CT. In select cases of
large intraventricular tumors, angiography may be
helpful to provide more detailed information
about the vascular supply of the lesion [3] and to
provide the opportunity for preoperative embolization. The absence of either large dural feeding
vessels or dedicated tumor vessels often makes
superselective embolization impossible, however,
and risks injury to the anterior and posterior
choroidal arteries supplying eloquent areas of the
brain.
A number of adjuncts for preoperative and
intraoperative planning that have facilitated removal of intraventricular lesions previously
thought to be resectable only with considerable
morbidity have become available during the last
several years [14,15]. Frameless stereotactic guidance systems allow precise localization of the
tumor, which permits the surgeon to choose an
approach to the lesion that minimizes manipulation of functionally critical cortex, targets vascular
pedicles, and potentially increases the safety of
aggressive tumor removal. Critics of these systems
argue that the accuracy and resolution of tumor
position and size are compromised if the brain is
retracted, diuretics are used, or the brain shifts
with tumor removal. The ventricular system,
however, is deep, based on the midline, and less
likely to shift than other areas of the cortex [14,15].
Moreover, technical developments linking stereotactic systems with real-time intraoperative ultrasound may improve accuracy and curtail these
515
516
517
Fig. 5. Surgical approaches to the lateral ventricles. The site of the skin incision (solid line) and the bone ap (broken
line) are shown for each approach. The anterior part of the lateral ventricle may be reached by the anterior transcallosal,
anterior transcortical, and frontal approaches. The posterior routes to the lateral ventricle are the posterior transcallosal,
posterior transcortical, and occipital approaches. The inferior part of the lateral ventricle can be reached using the
frontotemporal and temporal approaches. Ant., anterior; Post., posterior. (From Rhoton AL, Jr. The lateral and third
ventricles [Fig. 5.21]. Neurosurgery 2002;51(4 Suppl 1):S20771; with permission.)
518
Fig. 6. T1-weighted axial (A) and sagittal (B) MRI without contrast demonstrates a large mass in the left lateral ventricle
that was resected via a frontal transcortical approach.
519
Fig. 7. (A) T1-weighted sagittal MRI with contrast demonstrates an enhancing mass in the posterior lateral ventricle
that was resected via a parietal transcortical approach. (B) Intraoperative photomicrograph demonstrating the surgeons
view of the exposed lateral ventricle and tumor. Note the use of three self-retaining retractors for optimal exposure.
520
521
522
Fig. 9. Illustration of the posterior transcallosal approach. (A) The sagittal view depicts the corridor of approach to the
posterior corpus callosum. (B) View into the lateral ventricle, showing route of entry. (From Abosch A, McDermott
MW, Wilson CB. Lateral ventricular tumors [Fig. 64.4]. In: Kaye AH, Black P, editors. Operative neurosurgery.
London: Churchill Livingstone; 2000. p. 799812; with permission.)
Fig. 10. (A) T1-weighted sagittal MRI with contrast demonstrates a large cavernous malformation throughout the body
and atrium of the lateral ventricle that was resected via a posterior transcallosal approach with the patient in the prone
position. (B) Intraoperative photomicrograph showing the surgeons view into the lateral ventricles from this approach.
Both ventricles were widely opened, and the septum was resected with the mass.
523
Fig. 11. Illustration summarizing approaches to tumors of the lateral ventricles and some of the potential attendant
neurologic decits. (From Abosch A, McDermott MW, Wilson CB. Lateral ventricular tumors [Fig. 64.4]. In: Kaye AH,
Black P, editors. Operative neurosurgery. London: Churchill Livingstone; 2000. p. 799812; with permission.)
524
[8]
[9]
Summary
[10]
[11]
[12]
[13]
[14]
[15]
[16]
References
[1] Delni R, Acqui M, Oppido PA, Capone R,
Santoro A, Ferrante L. Tumors of the lateral
ventricles. Neurosurg Rev 1991;14:12733.
[2] Gokalp HZ, Yuceer N, Arasil E, Deda H, Attar A,
Erdodan A, et al. Tumours of the lateral ventricle.
A retrospective review of 112 cases operated upon
19701997. Neurosurg Rev 1998;21:12637.
[3] Pendl G, Ozturk E, Haselsberger K. Surgery of
tumours of the lateral ventricle. Acta Neurochir
(Wien) 1992;116:12836.
[4] Vogel S, Meyer R, Lehmann R, Woiciechowsky C.
Transcallosal removal of lesions aecting the third
ventricle: an anatomic and clinical study. J Neurosurg 1995;83:9235.
[5] Shucart WA, Stein BM. Transcallosal approach to
the anterior ventricular system. Neurosurgery
1978;3:33943.
[6] Apuzzo ML, Chikovani OK, Gott PS, Teng EL, Zee
CS, Giannotta SL, et al. Transcallosal, interforniceal
approaches for lesions aecting the third ventricle:
surgical considerations and consequences. Neurosurgery 1982;10:54754.
[7] Koos WT, Miller MH. Tumors of the ventricular
system. In: Koos WT, Miller MH, editors. In-
[17]
[18]
[19]
[20]
[21]
[22]
525
* Corresponding author.
E-mail address: jnb2@columbia.edu (J.N. Bruce).
cell tumors, and glial cell tumors [8,9]. A continuum of histopathologic subtypes exists within each
of these categories, ranging from benign to highly
malignant. Additionally, tumors of germ cell
origin may exhibit a mixture of cell types. Rarely,
miscellaneous lesions, such as metastatic tumors
[10,11], lymphoma [10], chemodectoma [12], and
primary melanocytic tumors [13], present in the
pineal gland. Benign pineal cysts are being
discovered with increasing frequency as people
undergo MRI scans for routine neurologic complaints [14].
In addition to tumors of the pineal gland,
posterior third ventricular tumors include thalamic astrocytomas and ependymomas, choroid
plexus tumors [1517], epidermoids [18,19], craniopharyngiomas [20], and meningiomas arising
from the velum interpositum [2123]. Large
falcotentorial meningiomas that extend anteroventrally may also impinge on the third ventricle
[24]. Arachnoid cysts [25], arteriovenous malformations [26], vein of Galen malformations [27,28],
and cavernous malformations [29,30] may also be
found in the region.
Aims of the operation
The goals of surgery for posterior third
ventricular tumors vary depending on the clinical
circumstances of an individual situation. The rst
objective is to establish a histologic diagnosis
[3134]. Surgery may be avoided altogether in the
presence of either serum or cerebrospinal uid
(CSF) a-fetoprotein or b-human chorionic gonadotropin. These markers are pathognomonic
for malignant germ cell elements; aected patients are treated nonoperatively with radio- and
1042-3680/03/$ - see front matter 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S1042-3680(03)00061-5
528
529
Fig. 1. Midsagittal gadolinium-enhanced MRI. (A) Germinoma with dorsal displacement of internal cerebral veins. (B)
Pineocytoma with dorsal displacement of internal cerebral veins. The two lesions are best approached via the
infratentorial-supracerebellar or occipital-transtentorial corridor. (C) Epidermoid tumor with ventral displacement of
the internal cerebral veins. This lesion is best approached via the posterior-interhemispheric corridor. (D) Tectal glioma
with extension into the cerebellomesencephalic ssure. The occipital-transtentorial approach provides the best trajectory
to the inferior pole of the tumor.
the mammillary bodies. The posterior third ventricle is bounded by a roof, a oor, two lateral
walls, and a posterior wall. The oor is formed by
mesencephalic structures extending between the
mammillary bodies and the cerebral aqueduct.
Viewed from within the ventricle, this surface is
smooth and concave. The posterior oor overlies
the posterior perforate substance anteriorly and
the medial part of the cerebral peduncles and the
tegmentum of the midbrain posteriorly [48,49].
The lateral walls of the posterior third ventricle
are formed inferiorly by the posterior hypothalamus and superiorly by the thalamus. The
thalamic and hypothalamic surfaces are demarcated by the hypothalamic sulcus, an indistinct
groove that extends from the foramen of Monro
to the aqueduct of Sylvius. The massa intermedia
often projects into the upper half of the posterior
third ventricle (present in approximately 75% of
specimens). The stria medullaris thalami extend
from the foramen of Monro to the habenulae
along the superomedial surface of the thalamus
530
Fig. 2. Sagittal (A) and dorsal (B) views of the anatomy of the third ventricle and pineal region. (From Apuzzo MLJ.
Surgery of the third ventricle. 1st edition. Baltimore: Williams & Wilkins; 1987. p. 612; with permission.)
laterally, and the quadrigeminal plate and cerebellar vermis inferiorly [51,52].
The roof of the posterior third ventricle has
four layers: the fornices superiorly, two thin
membranous layers of tela choroidea, and a potential space between the layers of the tela
choroidea called the velum interpositum. The
body of the fornix is suspended from the body
of the corpus callosum by the septum pellucidum.
Posteriorly, the septum wanes and the body
divides into crura that are directly attached to
the undersurface of the corpus callosum. The
superior layer of the tela choroidea is attached to
the fornix. The inferior layer of the tela choroidea
is attached to the teniae thalami and the pineal
body. The velum interpositum harbors the internal cerebral veins and the medial posterior
choroidal arteries. Although usually a potential
space, it may occasionally communicate with the
quadrigeminal cistern. The choroid plexus of the
third ventricle is suspended from the inferior leaf
of the tela choroidea. The lateral margins of the
roof of the third ventricle are composed of the
choroidal ssure superiorly and the stria terminalis thalami inferiorly [51,53,54].
Two of the three principal approaches to the
posterior third ventricle pass through the posterior incisural space. This anatomically complex
area is synonymous with the terms pineal region
and quadrigeminal cistern. The posterior incisural
space has an anterior wall, a roof, a oor, two
lateral walls, and a posterior apex at the level of
the tentorium. The superior portion of the
anterior wall is formed by the habenular trigone,
the habenular commissure, and the pineal body.
The pineal body overlies the quadrigeminal plate
of the midbrain, which is formed by the paired
superior and inferior colliculi. Inferiorly, the
anterior wall is formed by the lingula of the
vermis in the midline and the superior cerebellar
peduncles laterally. The roof of the posterior
incisural space is formed by the splenium of the
corpus callosum, the crus of the fornix, and the
hippocampal commissure. The oor of this space
is delimited by the anterior-superior surface of the
cerebellum. This space extends inferiorly into the
cerebellomesencephalic ssure. Each lateral wall is
formed by the pulvinar anteriorly, the crura of the
fornices, and the medial surface of the cerebral
hemisphere posteriorly. At the tentorial apex, the
quadrigeminal cistern is separated from the
superior cerebellar cistern by a thick sheet of
arachnoid that contains the precentral cerebellar
vein [2,52,55,56].
531
The posterior incisural space houses the conuence of the deep venous system. The paired
internal cerebral veins exit the velum interpositum
along the superolateral surface of the pineal body.
The union of the internal cerebral veins, forming
the great cerebral vein of Galen, may be located
above or posterior to the pineal body and inferior
or posterior to the splenium. The basal veins of
Rosenthal emanate from the ambient cisterns and
may be received either by the internal cerebral
veins or by the great vein directly. The precentral
cerebellar vein emanates from the cerebellomesencephalic ssure and drains directly into the vein of
Galen. It often receives the superior vermian vein.
After the great vein is formed, it ascends in
a superoposterior direction to join the straight
sinus at the falcotentorial junction [52,54,55].
Selection of a surgical approach
The typical pineal region mass is located in the
midline below the tentorial apex and displaces the
deep venous system superiorly. These anatomic
features give the infratentorial-supracerebellar
approach several natural advantages (Fig. 3)
[57]. The approach provides a midline trajectory
that is ventral to the velum interpositum and deep
venous system. The corridor between the cerebellum and the tentorium does not violate any
normal tissue, and the ability to use the sitting
position minimizes venous bleeding and facilitates
dissection of the tumor from the deep venous
system. In cases where the torcula is low lying or
the lesion has signicant lateral or supratentorial
extension, the occipital-transtentorial approach in
either the sitting or three-quarter prone position is
preferred. This approach provides the widest
exposure of both the supra- and infratentorial
compartments. This approach is also useful if
the tumor has signicant inferior extension into
the cerebellomesencephalic cistern; tumor in this
cistern would be out of view if approached via
the infratentorial corridor. For lesions that are
truly in the posterior third ventricle and do not
extend posterior to the splenium of the corpus
callosum, the interhemispheric-transcallosal approach in the lateral position is useful. This
approach follows the shortest route to the lesion
and has the advantage of working anterior to the
conuence of the deep venous system.
Patient positioning
Numerous patient positions have been described for approaching the pineal region and
532
Fig. 3. Principal approaches to the posterior third ventricle and pineal region. Midsagittal (A) and trajectory (B) views
along the operative corridors (arrows) provided by the occipital-transtentorial (1), infratentorial-supracerebellar (2), and
interhemispheric-transcallosal (3) approaches. (Adapted from Apuzzo MLJ. Surgery of the third ventricle. 1st edition.
Baltimore: Williams & Wilkins; 1987. p. 600; with permission. Adapted from Kaye A, Black P. Operative neurosurgery.
London: Churchill Livingstone; 2000. p. 832; with permission.)
533
Fig. 4. The sitting position. (From Kaye A, Black P. Operative neurosurgery. London: Churchill Livingstone; 2000.
p. 770; with permission.)
534
Fig. 5. (A) The lateral position with 30 of lateral exion. (B) Coronal section demonstrates retraction of the dependent
hemisphere by gravity and support of the opposite hemisphere by the falx. (Adapted from Apuzzo MLJ. Surgery of the
third ventricle. 1st edition. Baltimore: Williams & Wilkins; 1987. p. 6245; with permission.)
535
Fig. 6. The three-quarter prone position. (Adapted from Apuzzo MLJ. Surgery of the third ventricle. 1st edition.
Baltimore: Williams & Wilkins; 1987. p. 627; with permission.)
536
Fig. 7. The prone position. (From Kaye A, Black P. Operative neurosurgery. London: Churchill Livingstone; 2000.
p. 613; with permission.)
537
Fig. 8. Infratentorial-supracerebellar approach. (A) Numeric sequence of incisions of regional arachnoid; the precentral
cerebellar vein is sacriced to permit further relaxation of the anterior vermis. (B) Exposure of a pineal tumor with the
basal veins and vein of Galen overlying the mass. (C) View into the posterior third ventricle after tumor removal.
(Adapted from Apuzzo MLJ. Surgery of the third ventricle. 1st edition. Baltimore: Williams & Wilkins; 1987. p. 582, 586;
with permission.)
into the ventricle and cause obstructive hydrocephalus, shunt malfunction, or chemical meningitis. If necessary, long strips of Surgicel (Ethicon,
Somerville, NJ) draped over the surface of the
cerebellum and onto the tumor bed can provide
hemostasis at minimal risk.
After hemostasis is obtained, an attempt is
made to close the dura in a watertight fashion.
The craniotomy ap is secured with titanium
miniplates. As a general rule, the more complete
the dural and bone ap closure, the lower is the
risk of postoperative headaches and wound
538
Fig. 9. Options for tumor exposure using the posterior interhemispheric corridor. (A) Transcallosal approach. (B)
Retrocallosal and transsplenial variations employing a slightly more posterior trajectory. (Adapted from Apuzzo MLJ.
Surgery of the third ventricle. 1st edition. Baltimore: Williams & Wilkins; 1987. p. 617; with permission.)
539
540
Fig. 10. Occipital-transtentorial approach. The location of the deep venous system when the tumor arises form the
pineal gland (A), the falcotentorial junction (B), and the quadrigeminal plate (C). (Adapted from Apuzzo MLJ. Surgery
of the third ventricle. 1st edition. Baltimore: Williams & Wilkins; 1987. p. 603; with permission.)
541
542
Complications
Most patients display some degree of impairment of extraocular movements, particularly upgaze and diculty with convergence [81]. Any
preoperative oculomotor decit should be expected
to be exacerbated by the surgery. Fortunately,
these problems are generally transient and tend to
resolve over weeks to months. Permanent decits
are rare, although a mild limitation of upgaze may
persist, usually with limited clinical consequences.
Complications resulting from brain retraction
may occur with all three approaches. After the
supracerebellar-infratentorial approach, a brief
period of ataxia may occur. Rarely, cerebellar
infarction has been observed [74]. In the parietal
region, brain retraction during the transcallosal
approach may lead to contralateral sensory decits
or, rarely, to hemiparesis. Sacrice of bridging
veins may lead to cortical infarction, particularly if
more than one vein is taken. Occipital lobe
retraction during the transtentorial approach
may result in cortical visual eld decits [81].
543
544
[46]
[47]
[48]
[49]
[50]
[51]
[52]
[53]
[54]
[55]
[56]
[57]
[58]
[59]
[60]
[61]
[62]
[63]
[64]
[65]
[66]
[67]
[68]
[69]
[70]
[71]
[72]
[73]
[74]
[75]
[76]
[77]
[78]
545
[81] Nazzaro JM, Shults WT, Neuwelt EA. Neuroophthalmological function of patients with pineal region tumors approached transtentorially in
the semisitting position. J Neurosurg 1992;76:
74651.
1042-3680/03/$ - see front matter 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S1042-3680(03)00063-9
548
549
550
Fig. 2. (A) Endoscopic view of the oor of the third ventricle through the right foramen of Monro. The mamillary bodies
are prominences that serve as landmarks for the tuber cinereum, which lies anteriorly. The oor may be translucent as in
(C) or opaque as in (A). The ventriculostomy can be seen anterior to the left mammillary body, with the basilar artery
lying just underneath and visible through the fenestration. B, basilar artery; M, mamillary bodies. (B) Visualizing more
anteriorly, the ventriculostomy is now in the inferior view, with the infundibular recess seen anteriorly associated with
hypervascularity of the oor and the posterior aspect of the chiasm further anterior. C, chiasm; I, infundibular recess. (C)
In another case, the oor of the third ventricle is translucent, allowing immediate visualization of basilar artery and
dorsum sella. B, basilar artery; D, dorsum sella. (D) After fenestration of the oor with bipolar cautery, a Fogarty
balloon is passed through the opening to dilate the opening. F, Fogarty balloon.
provides adequate drainage of uid during continuous irrigation. The main advantage to the
rigid beroptic endoscope is the light weight and
small size of the endoscope, because the images
are carried by beroptic cable to a camera
attached distant to the operative site. In essence,
the surgeon only holds the beroptic cable
traversing through a holder with a working and
irrigation channel and a beroptic light source.
The camera digitizes the images, which are then
processed for projection onto a television monitor
o of the surgical eld. The image quality is
dependent on the density of beroptic cables and
551
552
553
554
Table 1
Outcome from endoscopic colloid cyst resection in the modern era
Author
Year
No. cases
Extent of resection
Follow-up
Recurrence (time)
1998
13
7 years
None
Decq et al
1998
15
15.2
months
1 (18 months)
King et al
1999
12
2002
12
24.5
months
56
months
Gaab et al
Hellwig et al
2003
20
10 near-complete
resection
3 subtotal resection
12 aspiration of cyst,
coagulation of cyst wall
3 partial removal
10 gross total resection
2 subtotal resection
1 no resection/observation
8 gross total resection
3 near-complete resection
1 gross total resection
19 subtotal resection
(coagulation of cyst wall)
62
months
0 symptomatic
1 asymptomatic
(93 months)
1 requiring
reoperation
(72 months)
555
Fig. 3. (A, B) Preoperative sagittal precontrast T1-weighted and coronal uid attenuated inversion recovery (FLAIR)
images demonstrating the colloid cyst associated with hydrocephalus. (C, D) Postoperative imaging after endoscopic cyst
resection. No visible remnant of the cyst is seen, and the ventricles have decreased in size. The patients imaging studies
show no cyst recurrence at 24 months of follow-up.
556
[13]
[14]
[15]
[16]
[17]
[18]
[19]
[20]
[21]
[22]
[23]
[24]
[25]
[26]
[27]
[37]
[38]
[39]
[40]
[41]
[42]
[43]
557
Intraventricular meningiomas
Michael W. McDermott, MD, FRCSC
Departments of Neurological Surgery and Radiation Oncology University of California at San Francisco,
505 Parnassus Avenue, M-774, San Francisco, CA 94143, USA
Incidence
Meningiomas are the second most common
primary brain tumor in adults [1]. In a surgical
series reported by Cushing and Eisenhardt
(N = 295) [2], intraventricular meningiomas accounted for only 1.3% of the total. Guidetti and
Delni [3] found that over a 38-year period, only
22 (1.5%) of 1451 meningiomas were intraventricular: 20 in the lateral ventricles, 2 in the fourth
ventricle, and none in the third ventricle. In an
earlier extensive review of the published literature
up to 1986, Criscuolo and Symon [4] identied 400
intraventricular meningiomas and categorized
their location. Eighty percent of intraventricular
meningiomas occur in the lateral ventricles, 15%
1042-3680/03/$ - see front matter 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S1042-3680(03)00055-X
560
Table 1
Common sites for intraventricular meningioma
Ventricle
Site
Frequency
Blood supply
Lateral
80%
Third
Atrium >80%
(L > R)
Posterior
15%
Fourth
Midline
5%
AChA
PLChA
PMChA
PLChA
PICA
Pathology
In the series of Guidetti and Delni [3], 81% of
the meningiomas were broblastic. Meningotheliomatous and psammomatous variants reported
by others as case reports are less common. All three
types are classied as grade I tumors in the World
Health Organization scheme, with a low risk of
recurrence and nonaggressive clinical behavior [8].
The pathologic entities encountered in the lateral
ventricle also include choroid plexus papilloma,
ependymoma, and metastases. The author has
operated on three large meningiomas of the
atrium, which proved pathologically to be metastases from renal cell (1 case) and thyroid (2 cases)
carcinoma. Posterior third ventricular tumors that
may mimic meningiomas radiographically include
pineocytoma and teratoma, whereas choroid plexus papilloma, ependymoma, and hemangioblastoma should be considered in the fourth ventricle.
Clinical presentation
Meningiomas of the lateral ventricles present
primarily with signs of increased intracranial
pressure. Headache, nausea/vomiting, and disturbance of vision are seen in 40% to 80% of patients
Fig. 1. Posterior coronal section of hemispheres. Note position of optic radiations lateral and inferior to atrium. Large
arrow on left indicates trajectory with posterior parietal-occipital approach. SPL, superior parietal lobule; IPL, inferior
parietal lobule; STG, superior temporal gyrus; MTG, middle temporal gyrus; ITG, inferior temporal gyrus; S, splenium;
OR, optic radiations.
80%
40%
35%
30%
25%
20%
20%
15%
60%
50%
50%
40%
20%
561
detail compared with CT scans, with meningiomas being iso- or hypointense on T1-weighted
images and T2-weighted images [10]. It is the
authors experience that broblastic meningiomas,
the most common pathologic subtype of meningioma within the ventricle, are usually prominently
hypointense on T2-weighted images. On T1weighted postcontrast images, there is uniform
contrast enhancement. Thin-cut axial spoiled
gradient recall (SPGR) images are routinely used
for image-guided surgical systems, and these
images can also provide the opportunity for
creating two-dimensional (2D) and three-dimensional (3D) venograms to assist the surgeon with
the preoperative selection of surgical approaches
[11]. The dierential diagnoses for other tumors in
these locations also need to be considered, especially as they relate to patient age (Table 3) [9].
MRI also oers the ability to perform spectroscopy
and blood volume time intensity maps, which,
alone or together, may increase the certainty of the
radiologic diagnosis. A high alanine-to-creatinine
ratio has been reported as a relatively specic MR
spectroscopy nding for meningiomas [10].
Cerebral angiography is rarely used these days,
because intraventricular meningiomas can rarely
be embolized. Angiography can conrm the predominant blood supply (see Table 1) and the
position of prominent parasagittal draining veins
[12]. Catheter cannulation of choroidal arteries is
dicult, however, and the target for occlusion is
distal in the vascular territory. Catheter-related
arterial spasm of the anterior choroidal artery
after attempted embolization of a vascular malformation, with subsequent infarction, has prevented us from considering preoperative embolization.
Thus, in selecting an operative approach, the
surgeon may need to take into consideration the
potential advantage of occluding the arterial blood
supply to large tumors early in the operation.
562
563
Table 3
Common radiographic dierential diagnoses for intraventricular meningioma
Site
Child
Adult
Atrium
CPP
Ependymoma
Metastasis
Ependymoma
Posterior
third
ventricle
Germinoma
Pineocytoma
blastoma
Astrocytoma
Teratoma
Pineocytoma
Astrocytoma
Metastasis
Fourth
ventricle
CPP
Ependymoma
Astrocytoma
Medulloblastoma
Ependymoma
CPP
Hemangioblastoma
Medulloblastoma
Radiosurgery
Radiosurgery is an eective form of treatment
for selected meningiomas, including intraventricular meningiomas. Reports on the tumor control
rates achieved with radiosurgery of 85% to 98%
cannot be overlooked during the informed consent process [14,15]. A size limit of 3 cm as used
in other brain locations is not acceptable for intraventricular tumors of the atrium, third ventricle, or fourth ventricle at our institution, and we
usually limit tumors to 1.5 to 2.0 cm depending on
associated vasogenic edema. Of the 13 patients
treated at our institution, 4 have had radiosurgery
for their intraventricular meningioma. In 2
patients, the treatment was used for residual or
recurrent disease, and in 2 patients, it was used as
the primary form of therapy. Of these latter 2
patients, 1 developed symptomatic subependymal
radiation toxicity despite a low marginal dose of
12 Gy. In spite of the fact that the tumor
Fractionated 3D conformal radiotherapy offers eective tumor control for residual or recurrent meningiomas in a variety of intracranial
locations [16]. Although there is no reason to
believe that the results should be any dierent for
intraventricular meningiomas, none of the four
patients at our institution who required additional
therapy other than initial surgery (ie, reoperation,
radiosurgery) was treated with this method.
Although the conformality of present-day techniques limits the volume of normal tissue irradiated,
intraventricular meningiomas are such discrete
targets that surgery or radiosurgery has been
recommended instead.
Surgical considerations
Evaluation of the patient for surgery involves
consideration of patient factors, such as age,
medical conditions, and neurologic status, and
b
Fig. 2. (A) Larger intraventricular meningioma presenting with seizure and language disturbance. Trapped temporal
horn (B) and evidence of parietal vasogenic edema (C). (D, E) Postoperative scans showing complete resection using
contralateral interhemispheric approach.
564
tumor characteristics, such as relation to symptoms and signs, growth rate, and resectability [7].
The routine evaluations for patients and specic
discussion of the approaches for intraventricular
meningiomas are reviewed below.
Preoperative studies
MRI without and with contrast is the basic
imaging study needed. For all supratentorial
approaches to the lateral ventricles, we obtain
volumetric, thin-cut, T1-weighted images for use
with image-guided surgical systems. MR venography can also be obtained to look at collapsed
vertex views or 3D reformats. Cerebral angiography is not routinely done, because the blood
supply to tumors in various locations is known
and embolization is not possible without signicant risk.
For patients having a transcallosal procedure
or transcortical superior parietal lobule approach
in the dominant hemisphere, the author always
obtains preoperative neuropsychologic testing as
a baseline. This can be repeated 3 months after
surgery and helps with quantitating patient status
for return to work and other disability issues. It
can also be used to document improvement in
function for patients who undergo successful
resection of large tumors or those associated with
hydrocephalus. If a superior parietal occipital
approach is selected, most anesthesiologists want
the patient to have a bubble echocardiogram done
to rule out a patent foramen ovale and a potential
right-to-left shunt. Humphrey visual elds should
be tested in all patients with tumors of the atrium.
General intraoperative measures
Patients undergoing transcortical approaches
receive anticonvulsants for 1 week around the
operation, beginning the morning of surgery.
Intravenous fosphenytoin or phenytoin is used
to load those patients who are not already on
medications. Standard measures to reduce intracranial pressure and improve blood rheology
are used. Mean arterial blood pressure should be
kept in the normal range, and hyperventilation
should be avoided throughout the case. Depending on the tumor location and hemisphere, every
attempt is made to identify and interrupt the
blood supply to these tumors early on.
Specic surgical approaches
Common surgical approaches by tumor site
are outlined in Table 4.
Middle temporal gyrus approach
Patient position: semilateral
Head position: extended on neck, tilted 20
downward, rotated 90 to opposite side,
parallel to oor
The middle temporal gyrus approach (Fig. 3) is
best suited for meningiomas of the atrium of the
lateral ventricle of the nondominant hemisphere.
A variety of skin incisions can be used, including
U-shaped incisions, reverse question mark incisions, or curvilinear incisions with posterior
hockey stick extension coming o at right angles
posteriorly [9,17]. A transsulcal approach in the
posterior third of the temporal lobe minimizes
tissue disruption, and image-guided systems can
be used to dene the precise trajectory to the
ventricle. The advantage of this approach is the
ability to pick up the anterior choroidal artery
within the temporal horn and eliminate the
predominant blood supply before tumor resection
starts. The choroid plexus can be followed back to
the tumor; internal debulking is then used,
followed by capsular dissection of the tumor from
the walls and oor of the ventricle. The surgeon
should be mindful of visual bers that rst pass
over the roof of the temporal horn and then swing
back lateral and inferior to the atrium in the
periventricular white matter; thus, horizontal
Table 4
Common surgical approaches by tumor site
Site
Approach
Patient position
Atrium
1. Left lateral
2. Supine or lateral
3. Lateral, tumor side up
4. Supine
1. Prone or semisitting
2. Prone, approach side down
Prone or concord
565
Fig. 3. Coronal section through atria, anterior cut to Fig. 1. White arrow marks middle temporal transsulcal or middle
temporal gyrus approach. Ipsilateral interhemispheric transcallosal approach (shaded arrow) and contralateral
interhemispheric transcallosal approach (solid arrow) are also marked. SF-P, Sylvian ssure, posterior; OTG,
occipital-temporal gyrus; PHG, parahippocampal gyrus; CC, corpus callosum; A, atrium; STG, superior temporal
gyrus; MTG, middle temporal gyrus; ITG, inferior temporal gyrus.
566
c
Fig. 4. (A) Axial T1-weighted image showing trapped temporal horn in patient treated with radiosurgery for atrial
meningioma 20 months earlier. In spite of reduction in tumor size, the patient developed a subependymal reaction
causing obstructive hydrocephalus. (B) Relatively small contrast-enhancing tumor at atrium. Immediate postoperative
scans with contrast showing reduction in size of temporal horn (C) and complete resection of tumor in axial (D) and
coronal (E) planes. (F) Contralateral interhemispheric transcallosal approach was used.
567
568
569
Intraventricular gliomas
Aaron S. Dumont, MDa,b, Elana Farace, PhDc, David Schi, MDd,
Mark E. Sharey, MDe,*
a
Gliomas remain the most common symptomatic primary brain tumor in adults. Those arising
within or relating to the ventricular surface
represent a relatively small but important proportion of all gliomas. Intraventricular gliomas
comprise a unique spectrum of histologic subtypes, the most common of which include
ependymomas, subependymomas (SEs), and subependymal giant cell astrocytomas (SEGAs).
Other less common variants, including chordoid
glioma [13], glioblastoma multiforme [4,5], and
mixed glial-neuronal tumors [6] among others,
have been reported. Each type of intraventricular
glioma has its own unique epidemiologic, clinical,
radiologic, and pathologic characteristics. Furthermore, each type commands its own constellation of management considerations, and each is
associated with dierent prognostic indicators and
outcomes.
Considerable advances have been made in the
contemporary understanding and management of
these tumors, particularly over the last several
decades. The advent and widespread implementation of advanced microsurgical technique coupled
with advancements and renements in adjuvant
therapies and their indications for use have helped
to improve the care of patients harboring these
* Corresponding author.
E-mail address: mes8c@virginia.edu (M.E. Sharey).
Ependymomas
Intracranial ependymomas refer to tumors of
neuroepithelial tissue arising from ependymal cells
lining the cerebral ventricles or from rests of
ependymal cells situated in the cortical white
matter. They occur most commonly in children
and young adults. Contemporary perspectives on
ependymomas have evolved considerably since the
original reported description of tumors of ependymal cell origin by Virchow [11] in 1863.
General comments and epidemiology
Ependymomas comprise between 3% and 10%
of intracranial tumors in most series [1217]. The
estimated incidence of ependymomas is approximately 0.2 to 0.8 per 100,000 persons per year
[14,18]. The median age at diagnosis is between 3
and 8 years [19,20], with 70% to 80% occurring in
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doi:10.1016/S1042-3680(03)00062-7
572
573
Fig. 1. Grade III ependymoma. (A) Head MRI reveals ventricular dilatation and an intensely enhancing mass in the
third ventricle on T1-weighted images after intravenous gadolinium administration. (B) The patient had evidence of
spinal drop metastases at the time of diagnosis (arrow).
574
575
576
577
578
ascribed to additional diculty in complete removal because of intimate association with lower
cranial nerves and critical vascular structures [34].
Miscellaneous factors. Although the aforementioned prognostic factors have been best characterized, several other factors have surfaced as
putative prognostic indicators. Gender, race, and
duration of symptoms have been suggested as
possible prognostic factors [19,99,100], although
they are questionable in their predictive ability.
Additional evidence is needed before any of these
latter factors could be considered a rm prognostic indicator.
Despite considerable progress in the management of patients with ependymomas, overall
prognosis remains relatively poor. Most patients
eventually develop recurrence, and the most
common site for recurrence seems to be the primary
tumor site [24,61,71,109]. Local recurrence remains
the primary cause of progressive neurologic decits
[24]. Tumor recurrence manifesting as metastases
without local recurrence is quite rare, occurring in
7% to 8% of cases [20,104]. The most important
factors in preventing recurrence are thus the same
factors as those for establishing local tumor
control. Unfortunately, treatment of tumor recurrence is quite limited. Repeat surgery is contemplated for local recurrence. Radiation therapy
has usually been administered; hence, further
radiation therapy is not usually an option. Chemotherapy may be administered, but its ecacy
has been disappointingly poor.
The overall 5-year survival rate for children
with ependymomas is approximately 39% to 93%
[19,20,22,34,36,37,99,101,104,111]. The 10-year
survival rates range from around 45% to 75%
[34,36,37,100,101]. The absolute survival rates do
not reveal the burden of neurologic morbidity
with which these patients may live. In children,
survivors have relatively low IQs and impaired
academic and psychosocial functioning that limit
their ability to interact with their environment.
Much of this morbidity may be iatrogenic, and
future eorts directed toward minimizing insult by
treatment on the developing nervous system are
clearly necessary.
Subependymomas
General comments and epidemiology
SEs are relatively uncommon well-dierentiated tumors associated with the ventricular
depends on the mode of presentation. Symptomatic patients usually present with obstructive
hydrocephalus and tend to be younger (average
age of 40 years) as opposed to those asymptomatic
patients who present at an average age of 60 years
[123]. It is estimated that 40% of SEs become
symptomatic [124]. Most asymptomatic patients
have lesions that are discovered on imaging studies
performed for clinical indications that are unrelated to the neoplasm (Fig. 2). Nevertheless, there
579
Fig. 2. Subependymoma. This patient originally presented with progressive ataxia. (A) Initially, the symptoms were
attributed to the mildly enhancing fourth ventricular mass (white arrow). (B) On closer inspection, the patient had
signicant spinal cord signal abnormality and myelomalacia (white arrow) related to basilar invagination and an
occipitalized atlas (black arrow). (C ) The patient underwent posterior fossa and C-1 decompression, total resection of the
subependymoma, and fusion.
580
581
Fig. 3. Subependymoma. (A) A nonenhancing lesion in the right frontal horn of the lateral ventricle. (B) The mass has
signicant hyperintensity on gradient echo MRI. (C ) An unusual appearance of intense homogeneous enhancement after
administration of intravenous gadolinium.
SE have been reported, including rhabdomyosarcomatous dierentiation, sarcomatous proliferation of the vasculature, and the presence of
melanin pigment [134136]. When mixed with
ependymoma, these areas reveal true or pseudorosettes, increased vascularity, increased mitosis,
necrosis, and increased cellularity [129].
Treatment and prognosis
Surgery
Advances in microsurgery have improved
surgical outcomes. One study compared the
582
Fig. 4. Subependymoma. (A) Isointense mass on T1-weighted images in patient with headaches and nystagmus. (B)
Minimal enhancement after intravenous gadolinium administration. (C ) Total resection was achieved despite broad
attachment to the oor of the ventricle.
583
584
Fig. 5. Subependymal giant cell astrocytoma. Axial (A) and coronal (B) MRI after intravenous gadolinium
administration demonstrating an intensely enhancing mass expanding the atrium of the left lateral ventricle. This
patient did not have stigmata of tuberous sclerosis.
Pathology
SEGAs are usually well-demarcated lobulated
masses that often appear calcied. Even macroscopically, cysts are not uncommon. SEGAs may
be quite vascular and have a reddish or hemorrhagic appearance. An origin of SEGA from
subependymal tubers has been postulated and
supported by the observations that there are
transitional lesions between tubers and SEGA
and that serial imaging studies have demonstrated
transformation of subependymal nodules into
symptomatic tumors [148150]. Current molecular studies suggest that SEGAs are the neoplastic
counterpart in the spectrum of central hamartomatous tuberous sclerosis complex lesions [151].
neuroendocrine dierentiation, perhaps to a greater extent than other mixed glial-neuronal neoplasms [151]. The Ki-67 labeling index (MIB-1) is
usually low [155].
Treatment and prognosis
Surgery
The surgical management of SEGAs is based
on the patients symptoms and the serial changes
on neuroimaging. An asymptomatic patient with
minimal changes in tumor size may be followed
expectantly with serial imaging. Patients who have
symptomatic obstructive hydrocephalus require
surgical intervention. In this instance, the treatment of choice is surgical resection, with gross
total resection as the surgical goal. In the past,
unilateral or bilateral shunting without tumor
resection has been advocated. With advances in
neuroanesthesia and microsurgery, however, these
lesions may be approached safely through transcallosal or transcortical transventricular approaches with acceptable morbidity as described
previously. Gross total tumor resection and
ventriculoventriculostomy (fenestration of the
septum pellucidum) may obviate the need for
shunting. It is recommended that neonates undergoing surgical treatment have preoperative
cardiac clearance because there is a reported
incidence of cardiac rhabdomyomas of tuberous
sclerosis complex, resulting in potential fatal
arrhythmias [156].
The frequency of tumor recurrence is low, with
a 10-year survival rate of nearly 80% after
surgical treatment [141]. Long-term survival is
possible, even after subtotal resection, because of
the limited growth potential of remaining tumor.
Because of the fact that rapid tumor regrowth is
reported [157], however, yearly follow-up MRI
should be obtained to monitor for tumor regrowth and hydrocephalus.
Radiation and chemotherapy
There is no signicant experience of the
treatment of SEGA with radiation therapy or
chemotherapy, although radiation can be considered in the rare setting of malignant degeneration.
The treatment of choice in the primary and
recurrent settings remains surgery.
585
Summary
Gliomas are the most common primary brain
tumor in adults, and those within or relating to
the ventricular surface represent a less common
but important subcategory. The most common
intraventricular gliomas include ependymomas,
SEs, and SEGAs. Other less common varieties
have been reported, including chordoid gliomas,
glioblastoma multiforme, and mixed glial-neuronal tumors. Each type of intraventricular glioma
is associated with its own unique constellation of
epidemiologic, clinical, radiologic, and pathologic
dening characteristics. Each tumor type has its
586
[9]
[10]
[11]
[12]
[13]
[14]
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[6] Jaeger M, Hussein S, Schuhmann MU, Brandis A,
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[8] Granzow M, Popp S, Weber S, Schoell B,
Holtgreve-Grez H, Senf L, et al. Isochromosome
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Stereotactic radiosurgery for recurrent ependymoma. Cancer 2000;88:8705.
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[103]
[104]
[105]
[106]
[107]
[108]
[109]
[110]
[111]
[112]
[113]
[114]
[115]
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594
Registry of Japan. Even less usual is the occurrence of a single intraventricular brain metastasis.
Schreiber and colleagues [28] reported that intraventricular metastases were found in 2.6% of
patients with cancer at autopsy but that single
intraventricular lesions were found in only 1
(0.14%) of the 737 cases analyzed.
Table 1 lists the cases of intraventricular
metastases reported in the literature. A total of 23
cases have been described, with the largest series
comprising 3 cases. Excluded from this list are cases
identied as being part of a larger series of intraventricular tumors [2224]; these cases are omitted
because the reports typically focus on the radiographic features or the surgical aspects of resecting
or biopsying intraventricular tumors in general and
not on the specics of metastatic lesions. For
example, Kelly and coworkers [30] reported on the
use of computer-assisted stereotactic surgery in the
removal of 58 intraventricular tumors, 3 of which
were intraventricular metastases; detailed descriptions of these cases were not given.
A search of the database of the Department of
Neurosurgery at the University of Texas M.D.
Anderson Cancer Center (M.D. Anderson) identied 35 patients (1.8%) with true intraventricular
metastases among a group of 1930 patients with
brain metastases treated during the 10-year period
between June 1993 and February 2003 (Table 2).
Thirty-one of the 35 patients had an intraventricular metastasis at the rst presentation of brain
metastasis, whereas 4 presented rst with an intraparenchymal metastasis and subsequently developed an intraventricular lesion. Of the 31 patients
with an intraventricular lesion at initial presentation, 12 (0.6% of all patients with metastases) had
single intraventricular metastatic tumors and 19
had multiple lesions (2 patients had 2 intraventricular lesions, 11 patients had 1 intraventricular
lesion and 1 intraparenchymal lesion, and 6 patients had 2 or more [range: 211] intraparenchymal lesions in addition to the intraventricular
lesion). This population undoubtedly represents
an underestimation of the total number of patients with intraventricular lesions seen at our
institution, because some patients with intraventricular metastases may have been treated by
medical or radiation oncologists without neurosurgical consultation.
Presentation and diagnosis
Patients with intraventricular metastases either
are known to have systemic cancer at the time of
595
ses, among which lung cancer typically predominates (30%60%), followed by breast cancer
(10%30%), melanoma (5%21%), and, less
commonly, renal cell carcinoma (1%2%) [31].
The high frequency of intraventricular renal
carcinomas suggests that there may be a tropism
of this tumor type for the ventricle, specically for
the choroid plexus, from which most of them
appear to arise. Matsumora and colleagues [15]
pointed out that renal cell carcinomas are divided
into two types: the rapidly progressive type,
which is often fatal within a few years of
diagnosis, and the slowly progressive type in
which patients survive for long periods with an
indolent tumor. The long time between diagnosis
of the brain metastases and the initial diagnosis of
renal cell carcinoma in the kidney that is common
in the cases reported in the literature suggests that
metastasis to the ventricle (choroid plexus) from
renal cell cancer is more prevalent in the slowly
progressive type. Taken together, these observations suggest that there may be a biologic basis for
the tendency of certain renal cell carcinomas to
metastasize to the ventricle. Notably, the kidney
and the choroid plexus function as plasma
ltering systems, and this suggests, albeit speculatively, that there may be a cellular and
molecular interaction between renal cell carcinoma and the cells comprising the choroid plexus
that is more important than simple mechanical
forces, such as blood ow. To our knowledge,
the nature of this interaction has not been
determined.
Location
In the literature (see Table 1) and our series
(see Table 2), intraventricular metastases were
observed to arise most commonly in the lateral
ventricle (68% of our cases), whereas metastases
to the fourth ventricle (21%) (Fig. 2) and the third
ventricle (11%) (Fig. 3) were less usual. Within the
lateral ventricle, the trigone was the most common site of metastasis (60% of our cases),
followed by the body (28%). In our series, 18 of
the 25 lateral ventricle tumors arose on the left
side, whereas there was less of a propensity for
localization in the dominant hemisphere in the
reports in the literature.
The distribution of intraparenchymal brain
metastases (ie, cerebral hemispheres [80%85%],
cerebellum [10%15%], and brain stem [3%5%])
roughly parallels the relative tissue and blood
volume of these regions of the brain. Using this
Reference (year)
[language if not
English]
596
Table 1
Published cases of intraventricular brain metastasesa
Time from
diagnosis
of primary Presenting
(months) symptoms
Bleeding/ No.
Location in
edema
metastases ventricle
Sex/age
(years)
Site of primary
tumor (pathology)
F/73
Decreased
consciousness
No/?
F/?
Breast (?)
No/+
Right trigone
?/?
Skin (melanoma)
Yes/+
Fourth ventricle
156
No/+
Left trigone
Approach not
specied
Parietal
craniectomy
F/55
Decreased
consciousness
Headache
60
Asymptomatic
Yes/+
Mertens et al
(1987)
[German]b
Shigemori et al
(1987)
[Japanese]b
Tanimoto et al
(1991)
Lung (poorly
dierentiated
epithelial
carcinoma)
?/newborn ? (neuroblastoma)
?/?
M/58
32
No/
M/64
Headache,
motor
weakness
Headache
No/+
F/59
48
Headache
No/+
Gastric (adenocarcinoma)
Decreased
Yes/
consciousness,
? seizure
Killebrew et al
(1983)
Mizuno et al
(1992)
Nakabayshi et al M/64
(1994)
Outcome
GTR, postoperative
bleed, reoperation;
required shunting;
alive at 4 years with
mild hemiparesis
Left trigone
Stereotactic
Radiation therapy to
biopsy
primary lung lesion;
died 2 months after
primary diagnosis
Right trigone
?
Received multiagent
chemotherapy; no
relapse at 2 years
Right body
Transventricular
Postoperative subdural
(near foramen)
hematoma; died on
postoperative day 3
Right trigone
Transcortical
Rapid regrowth
(middle
of tumor, progressive
temporal
hemiplegia; died
gyrus)
because of acute MI
before reoperation
Left lateral body Posterior
Tumor
interhemispheric
recurrence at 19
transcallosal
months, bedridden
Left center body Anterior
Died 2 months after
interhemispheric
surgery from
(transcallosal)
respiratory
complications
Healy and
Rosenkrantz
(1980)
Kendall et al
(1983)
Surgical
procedure
Suetake et al
(1994)
[Japanese]b
Berkow and
Kelly (1995)
M/78
Decreased
consciousness
Yes/+
Right trigone
F/13
Mediastinum (mixed 12
months
malignant germ cell
tumormature
teratoma)
Decreased
consciousness
No/?
Autopsy
Spetzger et al
(1995)
F/60
48
Headache,
nausea,
decreased
consciousness
Yes/
Fourth ventricle
(oor)
Suboccipital
craniotomy
Kohno et al
(1996)
M/45
Colon
(adenocarcinoma)
36
Hemiparesis,
aphasia,
hemianopsia
No/+
Left trigone
Transcortical
(parietal)
M/66
84
No/+
Right trigone
Interhemispheric
(transcallosal)
M/66
Lung (adenocarcinoma)
Hemiparesis,
disorientation
and memory
disturbance
Seizures
No/+
Right inferior
horn
Transcortical
(temporal)
Brandicourt et al ?/64
(1997)
[French]b
Matsumura
M/68
et al (1997)
Colon
(adenocarcinoma)
No/+
Third ventricle
84
Headache
No/
Right lateral
body
Raila et al
(1998)
F/47
Headache and
somnolence
Yes/+
Right trigone
Transventricular
via small
corticotomy
(frontal)
Approach not
specied
Arbelaez et al
(1999)
F/48
? (melanoma)
Headache
No/
Left trigone
Disease-free at
30 months
Death at 3 weeks
from secondary
complications
Partial resection,
no primary
found; stable
at 3 months
597
Transcortical
(temporal)
Received chemotherapy,
then presented with
brain herniation
from intraventricular
metastasis
Local rebleed on
postoperative day 2,
meningitis 1 week
after surgery,
recurrence at 18
months
Improved hemiparesis
and speech after gross
total resection,
followed by
postoperative
radiation therapy
Gross total resection,
postoperative
radiation therapy
Gross total resection,
postoperative
radiation therapy
Died 7 months later
598
1
No/
Headache
?
M/32
Iwatsuki et al
(1999)
[Japanese]b
Qasho et al
(1999)
Escott (2001)
Abbreviations: F, female; GTR, gross total resection; M, male; MI, myocardial infarction; , absent; +, present.
a
References were excluded for the reasons indicated: [42] article written in German, unable to conrm; [26] article written in Italian, unable to rule out meningeal
carcinomatosis; [43] clearly meningeal carcinomatosis; and [25] clearly meningeal carcinomatosis.
b
English abstract only.
Transcortical
(temporal)
Gamma Knife
radiosurgery
1
Bladder
(adenocarcinoma)
? (melanoma)
M/40
No/+
Right lateral
ventricle
Left trigone
Improved condition?
Approach not
specied
Left trigone
1
Yes/+
Headache,
decreased
consciousness
Seizure
Kidney (renal cell
carcinoma)
F/75
Bleeding/
edema
Site of primary
tumor (pathology)
Sex/age
(years)
Reference (year)
[language if not
English]
Table 1 (continued)
Time from
diagnosis
of primary
(months)
Presenting
symptoms
No.
metastases
Location in
ventricle
Surgical
procedure
Outcome
599
Table 2
Summary characteristics of patients at the University of Texas M.D. Anderson Cancer Center with intraventricular
metastases
Single metastasis
Number of patientsa
Age (years)
Sex
Intraventricular metastases
Presentation (with respect
to primary)
Time from diagnosis of
primary (months)
Presenting symptomsb
Hemorrhage
Location in ventriclec
12
64
9
3
12
0
1
Median (range)
Male
Female
At presentation
Developed subsequently
Synchronous
Metachronous
Median (range)
(34%)
(3669)
(75%)
(25%)
(100%)
(0%)
(8%)
11 (92%)
30.5 (084)
Headache
Nausea and vomiting
Decreased consciousness
Ataxia
Seizure
Motor weakness
Other
Asymptomatic
Lateral
Trigone
Body
Anterior horn
Temporal horn
Third
Fourth
Left
Right
Breast (adenocarcinoma)
Colon (adenocarcinoma)
Esophagus (adenocarcinoma)
Kidney (renal cell carcinoma)
Lung (adenocarcinoma)
(sarcoma)
Thyroid (papillary carcinoma)
Skin (melanoma)
6
2
1
2
0
1
3
2
2
10
(50%)
(17%)
(8%)
(17%)
(0%)
(8%)
(25%)
(17%)
(17%)
(27%)
8 (21%)
1 (3%)
0 (0%)
1 (3%)
1 (3%)
1 (3%)
8 (32%)
2 (8%)
1 (8%)
2 (17%)
0 (0%)
7 (58%)
1 (8%)
0 (0%)
0 (0%)
1 (8%)
Multiple metastases
23
50
12
11
19
4
1
(66%)a
(2069)
(52%)
(48%)
(83%)
(17%)
(4%)
22 (96%)
14.8 (022.8)
11
4
2
4
2
4
2
4
2
15c
(48%)
(17%)
(9%)
(17%)
(9%)
(17%)
(9%)
(17%)
(9%)
(40%)
7 (18%)
6 (16%)
1 (3%)
1 (3%)
3 (8%)
7 (19%)
10 (50%)
5 (10%)
4 (17%)
0 (0%)
1 (4%)
9 (39%)
2 (9%)
1 (4%)
2 (9%)
4 (17%)
Nineteen of the 23 patients with multiple metastases had an intraventricular metastasis at the initial diagnosis of
brain metastases, whereas 4 patients presented with an intraparenchymal metastasis and subsequently developed
intraventricular metastases.
b
Patient may have had more than one symptom.
c
Two patients had 2 intraventricular tumors (total number of tumors = 37).
600
Fig. 1. (A) Preoperative axial (left) and sagittal (right) contrast-enhanced MRI scans of a 50-year-old man with known
renal cell carcinoma and a left atrium/trigone lesion. (B) Corresponding immediate postoperative MRI scans after a gross
total resection via a transcortical superior lobule approach. The trajectory of this approach is evident.
Radiographic assessment
Radiographic assessments are used to determine the number of lesions and the size of each
lesion.
Single intraventricular metastases
Patients with single brain metastases are the
most appropriate surgical candidates. Indeed, two
independent prospective randomized studies demonstrated that surgical resection is superior to
WBRT in the treatment of single brain metastases
in terms of morbidity, recurrence, and overall
survival [35,36]. Although these studies did not
specically focus on intraventricular metastases, it
is probably appropriate to conclude that surgical
resection of intraventricular metastases is likely to
be superior to WBRT as long as the lesion can be
601
Fig. 2. (A) Preoperative axial (left) and sagittal (right) contrast-enhanced MRI scans of a 65-year-old woman with
known breast cancer and a fourth ventricular metastasis. (B) Corresponding immediate postoperative MRI scans after
a gross total resection via a midline suboccipital approach.
eective regardless of tumor size, whereas radiosurgery is not indicated for lesions greater than
3 cm in maximum diameter, and recent studies
suggest that good tumor control is achieved
primarily for lesions less than 1 cm in diameter
[38]. Thus, radiosurgery may be best applied to
relatively small lesions. Third, because surgery
rapidly removes the lesion, it reverses symptoms
more eciently than radiosurgery and reduces the
complications associated with long-term corticosteroid use. Fourth, surgery is eective regardless
of tumor histology, whereas the eects of radiosurgery are less predictable, particularly for
lesions that commonly produce ventricular metastases, such as renal cell carcinoma. Lastly, the
risk of delayed radiation injury that can occur
with radiosurgery is avoided with surgical intervention. The consequence of this injury may be
602
Fig. 3. Contrast-enhancing axial (A) and sagittal (B) MRI scans demonstrating a third ventricular tumor in a 42-yearold man with a history of thyroid cancer. The patient initially presented with an intraventricular hemorrhage that was
treated with ventricular drainage and a ventriculoperitoneal shunt. The images in this gure were obtained 2 months
after the initial presentation when the hemorrhage had resolved. The patient was treated with stereotactic radiosurgery
because of the deep location and small size of the lesion.
Surgical Techniques
Successful removal of intraventricular cerebral
metastases is safely accomplished in the modern
era because of advances in surgical technique.
Better understanding of the surgical anatomy of
these lesions has led to safer operative approaches, and accurate localization of lesions on
MRI has been translated into better intraoperative localization using image-guided methods.
Although the approaches to intraventricular
tumors have been discussed elsewhere in this issue
and certainly apply to metastatic tumors, certain
technical aspects unique to metastatic tumors are
worthy of note.
Surgical anatomy
Several anatomic features unique to intraventricular metastases must be recognized for safe
and eective resection. Intraventricular metastases are typically well-dened lesions that are
invariably attached to the choroid plexus. This
attachment may be quite broad, and inltration of
the choroid is common. Consequently, removal of
the choroid back to a zone that appears normal
may be required to prevent recurrence of the
lesion. In addition, attachment or inltration of
the wall of the ventricle may occur because of the
malignant nature of these tumors. Metastases
typically have a gliotic pseudocapsule, however,
603
604
Acknowledgment
The authors thank Weiming Shi for his
excellent assistance with the gures and Sandra
Flores for her excellent assistance with the
preparation of this manuscript.
References
[1] Healy JF, Rosenkrantz H. Intraventricular metastases demonstrated by cranial computed tomography. Radiology 1980;136(1):124.
[2] Kendall B, Reider-Grosswasser I, Valentine A.
Diagnosis of masses presenting within the ventricles
on computed tomography. Neuroradiology 1983;
25(1):1122.
[3] Killebrew K, Krigman M, Mahaley MS Jr, Scatli
JH. Metastatic renal cell carcinoma mimicking
a meningioma. Neurosurgery 1983;13(4):4304.
[20]
[21]
[22]
[23]
[24]
[25]
[26]
[27]
[28]
[29]
[30]
[31]
[32]
[33]
[34]
[35]
[36]
605
606
Division of Neurosurgery, Suite 1502, The Hospital for Sick Children, The University of Toronto,
555 University Avenue, Toronto, Ontario, Canada M5G 1X8
b
Division of Neuroradiology, The Hospital for Sick Children, The University of Toronto,
555 University Avenue, Toronto, Ontario, Canada M5G 1X8
Embryology
The neural crest begins to form at 3 weeks of
gestation of the trilaminar embryo (containing
ectoderm, mesoderm, and endoderm) by folding
of the neural plate into the neural tube. The
anterior neuropore closes at 18 days of gestation;
by the end of the fourth week, the primary brain
vesicle exists and the hindbrain exures outline
the primary divisions of the brain. The forebrain
divides into two secondary vesicles during the fth
week, forming the telencephalon and the diencephalon. With further invagination of the
telencephalon and growth of the cerebral hemispheres in a dorsorostral direction, the frontal,
temporal, and occipital poles form around the
diencephalon during the seventh week. The lateral
ventricles follow accordingly and are drawn from
the frontal pole to the temporal pole in a Cshaped manner.
A vascular layer of the pia mater fuses with the
ependyma to form the tela choroidea, which subsequently invaginates into the ventricles through
the choroidal ssure as the choroid plexus is
developing. Cerebrospinal uid (CSF) production starts, and, nally, in the second trimester,
the thin roof of the fourth ventricle bulges and
1042-3680/03/$ - see front matter 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S1042-3680(03)00057-3
608
Normal variants
Cavum septum pellucidum
As a single midline structure, the septum
pellucidum separates the two anterior horns of
the lateral ventricles (Fig. 1). The CSP is demarcated by the genu of the corpus callosum
anteriorly , by the columns and body of the fornix
posteriorly, by the body of the corpus callosum
superiorly, and by the rostrum of the corpus
callosum inferiorly. It consists of an ependymal
lining toward the ventricles and contains neuronal
and glial cell elements. These cell elements have
connections to the hypothalamus and the hippocampus. At birth, the two layers of the septum
pellucidum are separate and enclose a cavum.
Later in life, these two layers typically fuse into
a single septum. Autopsy and imaging studies
have shown that all premature infants and 97% of
term infants have a CSP, with the incidence
Fig. 1. Cavum septum pellucidum (CVP) and cavum velum interpositum (CVI). (A) Axial T2-weighted image
demonstrates the CVP anteriorly (arrow) and CVI posteriorly (open arrow). (B) Sagittal T1-weighted image demonstrates
anterior and superior displacement of the fornix (open arrow) distinguishing the CVI from the cavum vergae. Note
characteristic inferior displacement of the internal cerebral veins (arrow).
Intraventricular cysts
The development of intracranial cysts, especially those in the midline, can occur in isolation
or may be associated with further anomalies of the
brain, such as dysgenesis of the corpus callosum,
and incomplete forebrain cleavage within the
spectrum of holoprosencephaly [6,7]. Midline
cysts may be composed of ependymal, glioependymal, epithelial, neuroepithelial, or true arachnoid features.
There are several classication systems of
interhemispheric cysts, with most being based on
the communication with the ventricular system
and location in relation to brain parenchyma.
More recently, interhemispheric cysts have been
classied according to a combination of criteria,
including the presence of further developmental
anomalies or clinical characteristics [8,9].
Colloid cysts
Third ventricular colloid cysts constitute 0.5%
to 1% of all intracranial mass lesions and arise
from the anterior roof of the third ventricle. There
is a slight male predominance [10] and a pre-
609
610
Fig. 2. Colloid cyst. (A) Unenhanced CT in an adult demonstrates a homogeneous hyperdense lesion in the region of the
foramen of Monro. The ventricles are mildly enlarged. (B, C ) Coronal T1-weighted postcontrast and axial T2-weighted
images in another adult patient show a nonenhancing cyst with medium signal intensity in the roof of the third ventricle
causing mild ventricular enlargement. The unusual signal characteristics and location of the lesion are typical of a colloid
cyst.
611
Fig. 3. Intraventricular arachnoid cyst. (A) Coronal T2-weighted image demonstrates a large trigonal arachnoid cyst.
(B) Axial T2-weighted image demonstrates involvement of the perimesencephalic cistern via the choroidal ssure
(arrow), indicating a secondary rather than primary arachnoid cyst.
system as with suprasellar arachnoid cysts. Primary intraventricular arachnoid cysts are rare, with
less than 20 cases reported in the literature. Patients
typically present with symptoms of a spaceoccupying lesion and with headaches, delayed
psychomotor development, macrocephaly, hydrocephalus, and seizures [16,1923].
Symptomatic cysts are usually treated with
fenestration by endoscopic or open techniques
[1922,24]. During surgery, they are easy to differentiate from ependymal cysts because of their
sole adherence to the choroid plexus, whereas the
latter show attachment to the ventricular ependyma [25].
Choroid plexus cysts
Choroid plexus cysts are of neuroepithelial
origin and are most often found in the latera ventricles according to autopsy studies (Fig. 4). They
are a common nding on prenatal ultrasound
during the second trimester, but most spontaneously resolve by birth or early infancy. Ventricular
asymmetry may be attributed to a transient choroid plexus cyst that resolves later in life [26]. Only
a few reports exist of choroid plexus cysts that
612
Ependymal cysts
Pineal cysts
Fig. 5. Ependymal cyst. (A) Axial T2-weighted image demonstrates asymmetric lateral ventricles with deviation of the
septum pellucidum. (B) Axial T1-weighted postcontrast image conrms the presence of an intraventricular cyst with
cerebrospinal uid intensity with visualization of the posterior margin of the cyst wall (arrow). Imaging ndings are
consistent with an ependymal or arachnoid cyst.
613
614
Fig. 6. Pineal cyst. (A) Unenhanced CT scan shows a low-density lesion with thick rim calcications in the region of the
pineal gland. Coronal T2-weighted image (B) and sagittal T1-weighted postcontrast image (C) conrm the presence of
a nonenhancing cyst within the pineal gland. Note normal appearance of the third ventricle and lack of calcication
visualization on the MRI scan.
Fig. 7. Dandy-Walker malformation. Sagittal T2weighted image demonstrates the classic features of
a Dandy-Walker malformation. The vermis is severely
hypogenetic (arrow). The fourth ventricle is open,
communicating with a large retrocerebellar cyst. The
posterior fossa is enlarged with torcular-lambdoidal
inversion. Note the small occipital meningocele, a common association with Dandy-Walker malformations.
615
616
Fig. 8. Interhemispheric lipoma. (A) Sagittal T1-weighted image shows a hyperintense lesion surrounding a minimally
hypogenetic corpus callosum (absent splenium). (B) Coronal T1-weighted postcontrast image demonstrates
intraventricular extension (arrow), a common nding.
Fig. 9. Tuberous sclerosis. Axial T1-weighted postcontrast image demonstrates multiple enhancing and nonenhancing subependymal tubers. The large enhancing
lesion in the vicinity of the foramen of Monro (black
arrow) is consistent with a giant cell tumor. Mild
enlargement of the left lateral ventricle may be related
to intermittent obstruction of the foramen of Monro.
Note multiple periventricular parenchymal cysts (white
arrows), not uncommonly identied in tuberous sclerosis.
[7]
[8]
[9]
[10]
[11]
Summary
Intraventricular congenital lesions and colloid
cysts comprise a rather large spectrum of dierent
pathologic conditions. In most cases, treatment in
not warranted unless there is progressive ventricular obstruction with hydrocephalus or growth of
the lesion itself, making tissue biopsy and
histopathologic diagnosis necessary. Accordingly,
a precise neuroradiologic evaluation is of the
utmost importance, because most lesions, if not
symptomatic, only require clinical and radiologic
follow-up.
References
[1] Shaw CM, Alvord EC Jr. Cava septi pellucidi et
vergae: their normal and pathological states. Brain
1969;92(1):21323.
[2] Nakajima Y, Yano S, Kuramatsu T, Ichihashi K,
Miyao M, Yanagisawa M, et al. Ultrasonographic
evaluation of cavum septi pellucidi and cavum
vergae. Brain Dev 1986;8(5):5058.
[3] Kier LE. The evolutionary and embryologic basis
for the development and anatomy of the cavum veli
interpositi. AJNR Am J Neuroradiol 2000;21(3):
61214.
[4] Gangemi M, Donati P, Maiuri F, Sigona L. Cyst of
the velum interpositum treated by endoscopic fenestration. Surg Neurol 1997;47(2):1346.
[5] Gangemi M, Maiuri F, Godano U, Mascari C,
Longatti PL, Marzucco M. Endoscopic treatment
of para- and intraventricular cerebrospinal uid
cysts. Minim Invasive Neurosurg 2000;43(3):1538.
[6] Barth PG, Uylings HB, Stam FC. Interhemispheral
neuroepithelial (glio-ependymal) cysts, associated
[12]
[13]
[14]
[15]
[16]
[17]
[18]
[19]
[20]
[21]
617
with agenesis of the corpus callosum and neocortical maldevelopment. A case study. Childs Brain
1984;11(5):3129.
Mori K. Giant interhemispheric cysts associated
with agenesis of the corpus callosum. J Neurosurg
1992;76(2):22430.
Raybaud C, Girard N. Anatomic MRI study of
commissural agenesis and dysplasia of the telencephalon (agenesis of the corpus callosum and related
anomalies). Clinical correlations and morphogenetic interpretation. Neurochirurgie 1998;44(1 Suppl):
3860.
Barkovich AJ, Simon EM, Walsh CA. Callosal
agenesis with cyst: a better understanding and new
classication. Neurology 2001;56(2):2207.
Desai KI, Nadkarni TD, Muzumdar DP, Goel AH.
Surgical management of colloid cyst of the third
ventriclea study of 105 cases. Surg Neurol 2002;
57(5):295302.
Buttner A, Winkler PA, Eisenmenger W, Weis S.
Colloid cysts of the third ventricle with fatal outcome: a report of two cases and review of the
literature. Int J Legal Med 1997;110(5):2606.
Wiese JA, Gentry LR, Menezes AH. Bobble-head
doll syndrome: review of the pathophysiology and
CSF dynamics. Pediatr Neurol 1985;1(6):3616.
Maeder PP, Holtas SL, Basibuyuk LN, Salford LG,
Tapper UA, Brun A. Colloid cysts of the third
ventricle: correlation of MR and CT ndings with
histology and chemical analysis. AJR Am J Roentgenol 1990;155(1):13541.
Hopf NJ, Perneczky A. Endoscopic neurosurgery
and endoscope-assisted microneurosurgery for the
treatment of intracranial cysts. Neurosurgery 1998;
43(6):13306.
Di Rocco C, Di Trapani G, Iannelli A. Arachnoid
cyst of the fourth ventricle and arrested hydrocephalus. Surg Neurol 1979;12(6):46771.
Korosue K, Tamaki N, Fujiwara K, Matsumoto S.
Arachnoid cyst of the fourth ventricle manifesting
normal pressure hydrocephalus. Neurosurgery 1983;
12(1):10810.
Nakase H, Hisanaga M, Hashimoto S, Imanishi M,
Utsumi S. Intraventricular arachnoid cyst. Report
of two cases. J Neurosurg 1988;68(3):4826.
Yeates A, Enzmann D. An intraventricular arachnoid cyst. J Comput Assist Tomogr 1979;3(5):697
700.
Kurokawa Y, Sohma T, Tsuchita H, Kitami K,
Suzuki S, Ishikawa A. A case of intraventricular
arachnoid cyst. How should it be treated? Childs
Nerv Syst 1990;6(6):3657.
Martinez-Lage JF, Poza M, Sola J, Puche A.
Congenital arachnoid cyst of the lateral ventricles
in children. Childs Nerv Syst 1992;8(4):2036.
Nakase H, Ishida Y, Tada T, Sakaki T, Goda K,
Tunoda S, et al. Neuroepithelial cyst of the lateral
ventricle. Clinical features and treatment. Surg
Neurol 1992;37(2):94100.
618
[38]
[39]
[40]
[41]
[42]
[43]
[44]
[45]
[46]
[47]
[48]
[49]
[50]
[51]
[52]
[53]
[66]
[67]
[68]
[69]
[70]
[71]
[72]
[73]
[74]
[75]
[76]
619
Epidemiology
Choroid plexus papillomas (CPPs) and their
malignant counterpart, choroid plexus carcinomas
(CPCs), are uncommon, comprising only 0.5% to
0.6% of all brain tumors. They are encountered in
all age groups but are mainly seen in childhood.
There is a particular preponderance in infants and
extremely young children. In his review of all
published cases before 1974, Laurence [4] reported
that 45% presented in the rst year of life, whereas
74% occurred in the rst decade. Wol and
colleagues [5] studied all available cases reported
in the literature until 1998 and performed a detailed
meta-analysis. This report noted a male-to-female
ratio of 1.2:1, a median age at diagnosis of 3.5.
years, and a striking dierence in tumor location
and age. Supratentorial tumors (lateral and third
ventricles) occurred mostly in infants, with a median age of 1.5 years at diagnosis in this group. By
comparison, the median ages at diagnosis of
tumors in the fourth ventricle and cerebellopontine
angle (CPA) were 22.5 and 35.5 years, respectively.
Laurence [4] did note that 50% of cases reviewed
were situated in the lateral ventricles, 37% in the
fourth ventricle, 9% in the third ventricle, and the
remainder in other locations.
As expected, reviews from pediatric centers
report that a higher percentage (1.8%2.9%) of
their cases are choroid plexus tumors [68]. Two
reviews of tumors in the rst year of life by
Galassi et al [9] and Haddad et al [10] found that
choroid plexus tumors comprised 14% and 12.8%
of all cases, respectively. Most series noted
previously have not reported any predilection
for right or left ventricle or sex. CPCs comprise
29% to 39% of all choroid neoplasms [8,11,12].
1042-3680/03/$ - see front matter 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S1042-3680(03)00058-5
622
Pathology
Gross appearance and histopathology
Typically, most astrocytic tumors are soft and
poorly cohesive masses. In contrast, CPPs recapitulate the normal appearance of the choroid
plexus with well-dierentiated secondary structures, resulting in a cauliower-like appearance
(Fig. 1). The surface of the tumor is similar to the
soft fronds of normal choroid, with the overall
shape being roughly globular. The stroma can
be brous and tough in consistency. Evidence of
previous hemorrhage is sometimes apparent.
Because papillomas are benign, they tend to
expand the ventricle rather than invade adjacent
brain. Nevertheless, the proximity of these tumors
to deep-seated structures, such as the internal
cerebral veins and limbic structures, can make their
removal dicult.
The microscopic appearance of papilloma is
also similar to that of the normal choroid plexus.
There are many papillae covered with a simple
cuboidal or columnar epithelia. The stroma of
these brovascular structures is composed of
connective tissue and small blood vessels. The
presence of the connective tissue stroma is notable
mainly because it allows one to distinguish
between CPP and papillary forms of ependymoma
(whose stroma is composed of brillary neuroglia).
623
Clinical features
For most patients with a choroid plexus tumor,
hydrocephalus is responsible for the presenting
symptoms. It is caused by overproduction of CSF
and, in certain cases, the obstruction of CSF
pathways, although it seems that overproduction
is the major contributing factor (Fig. 2) [34].
Resolution of hydrocephalus has been reported
after complete tumor removal, suggesting that
CSF hypersecretion was responsible for ventriculomegaly [3537]. Variations are likely to exist,
because a normal rate of CSF production has been
reported in a patient harboring a papilloma [38].
The most common presentation of choroid
plexus neoplasms is related to increased intracranial hypertension secondary to obstructive hydrocephalus or CSF overproduction [4,8,39]. Because
most cases occur in infants and young children,
there are characteristic features of raised
624
Treatment
Overall objectives
GTR is associated with the most favorable
outcome in patients with choroid plexus tumors
[5,43]. Therefore, any treatment plan should be
tailored to reach this objective. The following steps are likely to be encountered as part of
the surgical plan: (1) temporary or permanent
625
Fig. 3. An 18-month-old girl with a large left ventricular choroid plexus papilloma. (A) T1-weighted image after
injection of gadolinium. (B) T2-weighted axial image. (C) MR angiogram demonstrating a choroidal artery arising from
the posterior cerebral artery leading to the tumor. (D) A postoperative, coronal, T1-weighted image demonstrating the
surgical route clearly shows an enlarged choroidal artery leading into the tumor. Postoperative coronal MRI
demonstrates the route through the temporal lobe used to access the feeding artery initially and then the actual tumor
itself.
626
627
628
Fig. 5. A 2-month-old child with a right atrial choroid plexus papilloma is shown. (A) The preoperative T1-weighted
image demonstrates bright enhancement after injection of gadolinium. (B) During the initial attempted resection, the
deeper vascular pedicle could not be secured; after two blood volumes had been lost, the procedure was stopped. The
postoperative CT scan demonstrates a large intraventricular and intraparenchymal hematoma. (C) After 2 weeks,
the patient was taken back to the operating room, where the residual tumor and hematoma were resected. Postoperative
CT shows resolution of the hydrocephalus with large extra-axial cerebrospinal uid collections bilaterally. The patient
did not require placement of ventriculo-peritoneal (VP) shunt.
629
As noted previously, the treatment of hydrocephalus goes hand in hand with the treatment of
choroid plexus tumors. One treatment-related
complication is the development of large subdural
collections. These can result from a transcortical
approach or by craniocerebral disproportion. In
the former, the cause is believed to be a persistent
ventriculosubdural stula. Boyd and Steinbok [53]
report that placement of pial sutures at the
conclusion of the procedure can prevent the development of subdural collections. In the latter,
reduction in the size of the ventricles and removal
of a large mass can lead to an enlarged calvarium
relative to the size of the brain (see Fig. 5C;
Fig. 6). At times, these subdural collections may
require treatment by the placement of a subduralperitoneal shunt.
Fig. 6. Persistent subdural collections in a child several
months after removal of a lateral ventricle choroid
plexus papilloma. The patient remained asymptomatic
with a gradual reduction in the size of the collections;
thus, surgical drainage was not required.
Summary
Choroid plexus tumors represent a well-dened
subset of brain tumors that occur mainly in young
children. Surgical resection for papilloma is
usually curative, although careful surgical planning is required to minimize the potential risks.
Although adjunctive therapy for carcinoma includes chemotherapy or radiation, the long-term
survival for carcinoma remains poor.
References
[1] Chow E, Jenkins JJ, Burger PC, Reardon DA,
Langston JW, Sanford RA, et al. Malignant
evolution of choroid plexus papilloma. Pediatr
Neurosurg 1999;31:12730.
[2] Davis LE, Cushing H. Papillomas of the choroid
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[3] Dandy W. Diagnosis, localization, and removal of
tumours of the third ventricle. Bull Johns Hopkins
Hosp 1922;33:1889.
[4] Laurence KM. The biology of choroid plexus
papilloma and carcinoma of the lateral ventricle.
In: Vinken PJ, Bruyn GW, editors. Handbook
of clinical neurology. New York: Elsevier; 1974.
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[5] Wol JE, Sajedi M, Brant R, Coppes MJ, Egeler
RM. Choroid plexus tumours. Br J Cancer 2002;87:
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[6] Sarkar C, Sharma MC, Gaikwad S, Sharma C, Singh
VP. Choroid plexus papilloma: a clinicopathological
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[7] Asai A, Homan HJ, Hendrick EB, Humphreys
RP, Becker LE. Primary intracranial neoplasms
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[24] Vajtai I, Varga Z, Aguzzi A. MIB-1 immunoreactivity reveals dierent labelling in low grade and in
malignant epithelial neoplasms of the choroid
plexus. Histopathology 1996;29:14751.
[25] Zwetsloot CP, Kros JM, Paz y Gueze HD. Familial
occurrence of tumours of the choroid plexus. J Med
Genet 1991;28:4924.
[26] Brinster RL, Chen HY, Messing A, van Dyke T,
Levine AJ, Palmiter RD. Transgenic mice harboring SV40 T-antigen genes develop characteristic
brain tumors. Cell 1984;37:36779.
[27] Marks JR, Lin J, Hinds P, Miller D, Levine AJ.
Cellular gene expression in papillomas of the
choroid plexus from transgenic mice that express
the simian virus 40 large T antigen. J Virol 1989;
63:7907.
[28] Bergsagel DJ, Finegold MJ, Butel JS, Kupsky WJ,
Garcea RL. DNA sequences similar to those of
simian virus 40 in ependymomas and choroid plexus
tumors of childhood. N Engl J Med 1992;326:
98893.
[29] Zhen HN, Zhang X, Bu XY, Zhang ZW, Huang
WJ, Zhang P, et al. Expression of the simian virus
40 large tumor antigen (Tag) and formation of Tagp53 and Tag-pRb complexes in human brain
tumors. Cancer 1999;86:212432.
[30] Arbeit JM, Munger K, Howley PM, Hanahan D.
Neuroepithelial carcinomas in mice transgenic with
human papillomavirus type 16 E6/E7 ORFs. Am J
Pathol 1993;142:118797.
[31] Sevenet N, Lellouch-Tubiana A, Schoeld D,
Hoang-Xuan K, Gessler M, Birnbaum D, et al.
Spectrum of hS.N.F5/INI1 somatic mutations in
human cancer and genotype-phenotype correlations. Hum Mol Genet 1999;8:235968.
[32] Sevenet N, Sheridan E, Amram D, Schneider P,
Handgretinger R, Delattre O. Constitutional mutations of the hS.N.F5/INI1 gene predispose to
a variety of cancers. Am J Hum Genet 1999;65:
13428.
[33] Rickert CH, Wiestler OD, Paulus W. Chromosomal
imbalances in choroid plexus tumors. Am J Pathol
2002;160:110513.
[34] Eisenberg HM, McComb JG, Lorenzo AV. Cerebrospinal uid overproduction and hydrocephalus
associated with choroid plexus papilloma. J Neurosurg 1974;40:3815.
[35] Wilkins RH, Rutledge BJ. Papillomas of the
choroid plexus. J Neurosurg 1961;18:148.
[36] Matson DD, Crofton FD. Papilloma of choroid
plexus in childhood. J Neurosurg 1960;17:100227.
[37] Gudeman SK, Sullivan HG, Rosner MJ, Becker
DP. Surgical removal of bilateral papillomas of the
choroid plexus of the lateral ventricles with
resolution of hydrocephalus. Case report. J Neurosurg 1979;50:67781.
[38] Sahar A, Feinsod M, Beller AJ. Choroid plexus
papilloma: hydrocephalus and cerebrospinal uid
dynamics. Surg Neurol 1980;13:4768.
[47]
[48]
[49]
[50]
[51]
[52]
[53]
631
April
July
October
A
Abdominal muscle strength, evaluation of, for
pain management, 342
Abscess, pituitary, imaging of, 71
N-Acetyl aspartate, in magnetic resonance
spectroscopy, in obsessive-compulsive
disorder, 214215
Acetylcholine modulators, intrathecal delivery of,
361
Acidophilic stem cell adenoma, 27
Acromegaly, in somatotroph adenoma, 4345
after pituitary surgery, 134, 139141
treatment of, 8283, 139141
Adenoassociated virus, for gene transfer, for pain
management, 423424
Adenohypophysis
anatomy of, 5557
histogenesis of, 13
histology of, 25
hormones of, 1720
vascular anatomy of, 1415
Adenohypophysitis, lymphocytic, imaging in,
7273, 75
Adenoma
acidophilic stem cell, 27
corticotropinsecreting. See Corticotroph
adenoma.
gonadotropinsecreting. See Gonadotroph
adenoma.
growthhormone secreting. See Somatotroph
adenoma.
mammosomatotroph, 2728
1042-3680/03/$ - see front matter 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S1042-3680(03)00089-5
634
B
Baclofen, intrathecal delivery of, 360361
Ballantine, H. Thomas, Jr., on psychiatric
neurosurgery, 308
Barber, Jesse, on psychiatric neurosurgery, 311
Behavioral therapy, for obsessive-compulsive
disorder, 200201
Benabid, Alim, deep brain stimulation studies of,
315
Bifrontal transbasal approach, to parasellar and
suprasellar lesions, 114119
Biopsy
in third ventricular tumors, 528
stereotactic, in neurocytoma, 494
C
Cabergoline, for hyperprolactinemia, in
prolactinoma, 8182, 90, 143
Calcication
in ependymoma, 473, 572
in subependymal giant cell astrocytoma, 584
in subependymoma, 475
Calcitonin gene-related peptide, in pain
transmission, 446, 452
Calcium, in pain transmission, 451452
Calcium channel blockers, intrathecal delivery of,
361
Califano, Joseph, psychiatric neurosurgery
regulations approved by, 314
Callostomy, for colloid cyst, 609
Camera, for endoscopic intraventricular surgery,
549550
Canavan disease, gene therapy for, 431
Cancer, metastasis from. See Metastasis.
Cancer pain
denition of, 330
intrathecal management of, 355, 359, 361,
383385
Capsule, internal, anterior limbs of, electrical
stimulation of, in obsessive-compulsive
disorder, 267274
Capsulotomy, for psychiatric disorders
depression, 219, 232233
history of, 191192
obsessive-compulsive disorder, 204208, 219,
232233
Carcinoma
choroid plexus
clinical features of, 623624
epidemiology of, 469, 621
imaging of, 624, 626
outcome of, 628629
pathology of, 469472, 622623
treatment of, 627628
pituitary, 3637
Cardiovascular disorders, in acromegaly, 44
Catheters, for intrathecal pain management,
356358, 382383
Cationic polymers, for gene transfer, 421
Cauda equina syndrome, in intrathecal
medication delivery, 358
Caudate nucleus
anatomy of, 511
connections to
in depression, 216219
in obsessive-compulsive disorder, 214,
218219
Cavernous sinuses
anatomy of, for pituitary surgery, 1617
exposure of, in pituitary surgery, 102
Cavum septum pellucidum, 608
Cavum veli interpositi, 609
Cavum vergae, 608609
Central neurocytoma. See Neurocytoma,
intraventricular.
Central sensitization, physiology of, 450452
Cerebellum, hypoplasia of, in Dandy Walker
malformation, 613615
Cerebral salt wasting, after pituitary surgery,
128
635
Cerebrospinal uid
analysis of, in depression, in vagus nerve
stimulation, 280
leakage of in intrathecal medication delivery,
358
Chemotherapy
for choroid plexus carcinoma, 627628
for ependymoma, 576577
for neurocytoma, 487, 501503
for subependymoma, 583584
Chlorpromazine, for psychiatric disorders, history
of, 190
Chlorpropamide, for diabetes insipidus, 132
Chondrosarcoma, clival, imaging of, 77
Chordoma, clival, imaging of, 7677
Choroid plexus
anatomy of, 512513
cysts of, 611612
tumors of
clinical features of, 623624
dierential diagnosis of, 472
epidemiology of, 469, 621
genetic features of, 472, 623
imaging in, 624, 626
in children. See Pediatric patients, choroid
plexus tumors in.
in third ventricle. See Third ventricular
tumors.
metastatic, 598
outcome of, 628629
pathology of, 469472, 621623
subependymal giant cell astrocytoma,
585586
treatment of, 624628
Chroman cells, intrathecal delivery of, 361
Cilia, in choroid plexus tumors, 470471
Cingulate cortex
anterior, connections to
in depression, 216219
in obsessive-compulsive disorder, 214,
218219
in nociception, 448
Cingulotomy, for psychiatric disorders, 191192,
225235
anatomic considerations in, 225227
complications of, 230
depression, 204206, 208209, 218, 225228
history of, 225
obsessive-compulsive disorder, 204206,
208209, 216, 225231
636
Cingulotomy (continued )
SPECT in, 238249
patient selection for, 227228, 231
physiologic considerations in, 225227
results of, 228231, 241243
technique for, 228, 239
versus alternative surgical methods, 231233
Clivus
exposure of, in pituitary surgery, 101102
neoplasms of, imaging of, 7678
Coccydynia, spinal cord stimulation for, 378
Corticostriatothalamocortical circuitry
in depression, 216219
in obsessive-compulsive disorder, 214,
218219
Corticotroph(s), 25
Colorectal cancer
in acromegaly, 45
intraventricular metastasis from, 596597
Corticotroph adenoma
Cushings disease in, 4548
diagnosis of, 169
long-term management of, 169
outcome of, 169
medical treatment of, 142
pathology of, 3132
persistent, 141142
radiotherapy for, 142
silent, 32
surgical treatment of, 169
persistent, 141142
postoperative endocrine management in,
130131, 133134
preoperative management in, 94
Corticotropin, 1819, 25
adenomas secreting. See Corticotroph
adenoma.
deciency of, after pituitary surgery, 132133
measurement of, in corticotroph adenoma, 47
Corticotropin-releasing hormone stimulation test,
in corticotroph adenoma, 47
Cortisol
excess of, in corticotroph adenoma, 3132,
4548
medical treatment of, 8384
persistent, 141142
preoperative management of, in pituitary
tumors, 94
Cranial nerve dysfunction, after pituitary
radiotherapy, 162
Craniopharyngioma. See also Third ventricular
tumors.
imaging of, 6163
637
Cushings disease
after pituitary surgery, 130131, 133134
in corticotroph adenoma, 3132, 4548
long-term management of, 169
outcome of, 169
persistent, 141142
medical treatment of, 8384
Depression, 199212
clinical features of, 201
epidemiology of, 201
evaluation of, before pain management,
411412, 414415
medical treatment of, 201
neurocircuitry models for, 216219
neuroimaging in, 216219
neurosurgery for
capsulotomy, 219, 232233
cingulotomy, 204206, 208209, 225228
current awareness of, 201202
eectiveness of, 207209
history of, 202203
limbic leukotomy, 205207, 209, 232
modern procedures for, 203205
neuroimaging and neurocircuitry related to,
218219
safety of, 205207
subcaudate tractotomy, 204205, 207, 218,
231
thalamocortical dysrhythmia in. See
Thalamocortical dysrhythmia.
transcranial magnetic stimulation for, 288293
maintenance, 292
meta-analysis of, 289292
with intentional seizure generation, 294295
treatment of, before pain management, 348
vagus nerve stimulation for, 275282
D
Dandy Walker malformation, 613615
Deep brain stimulation, 389399
complications of, 395396
for neuropathic pain, 390391
for nociceptive pain, 391
history of, 332334, 389390
mechanism of action for, 390391
o-label use of, 396
patient selection for, 391392
physiology of, 454455
preoperative evaluation for, 391392
rationale for, 390391
results of, 394396
target site selection for, 392
technique for, 392394
tolerance to, 395
638
Dopamine agonists
for gonadotroph adenoma, 84
for prolactinoma, 3031, 8182, 8990, 143
Dott, Norman, transsphenoidal approach of, 57
Dynorphin, in antinociception, 453
E
Edema, in intrathecal morphine delivery, 385386
Electrical stimulation
deep brain, 332334, 389399
implantable stimulators for, 331334, 372375,
403406
internal capsule anterior limb, for obsessivecompulsive disorder, 267274
motor cortex, 334
peripheral nerve, 331, 401408
precentral, 334, 437443
spinal cord, 331332, 365380
vagus nerve, for depression, 275282
Electroconvulsive therapy, for psychiatric
disorders, 184, 201
versus transcranial magnetic stimulation,
287289, 291292, 294
Electroencephalography, in depression, in vagus
nerve stimulation, 280
Electropermeabilization, in gene therapy, for
pain, 420
Eloquent cortex, localization of, transcranial
magnetic stimulation for, 287
Empty sella syndrome, 16, 6465
Endocrinopathies, in pituitary tumors. See
Pituitary tumors, endocrinopathies in.
Endometriosis, pain in, gene therapy for, 430431
Endonasal transsphenoidal approach, to pituitary
tumors, 99100
lateral (Hirsch), 12
Endoscopy
in intraventricular surgery, 547557
anatomic considerations in, 548549
colloid cyst resection, 552554
equipment for, 549551
history of, 547548
pineal tumor management, 554555
two-portal, 551
ventriculostomy, 551552
in transsphenoid approach, to pituitary
tumors, 104105
Enkephalins, in antinociception, 453
F
Federal Employees Compensation Act, 349
Federal Employers Liability Act, 349
639
Ganglioneurocytoma, 477
Gasserian ganglion, in nociception, 446447
Gate theory of pain, 328331, 451
Gemistocyte-like cells, in subependymal giant cell
astrocytoma, 475476
Gene-gun method, for gene transfer, 428
Gene therapy, for pain, 419435
advantages of, 419, 422
cell lines for, 429430
complications of, 430
ex vivo, 429430
neuromodulation, 425429
nonviral, 420421
root causes, 429430
viral vectors in, 421425
Germinoma, parasellar, imaging of, 71
Giant cell astrocytoma, subependymal, 470471,
475476, 584585
Glial-derived neurotrophic factor, in nociception,
446
Glial brillary acidic protein
for gene transfer, for pain management, 422
in choroid plexus tumors, 470
in ependymoma, 473
in subependymal giant cell astrocytoma, 585
Glioma. See also Ependymoma;
Subependymoma.
intraventricular
choroid, 585586
subependymal giant cell astrocytoma,
470471, 475476, 584585
types of, 571
parasellar, after pituitary radiotherapy, 162
Glutamate, in pain transmission, 451452
G
a-Galactosidase A deciency, pain in, gene
therapy for, 431
Gall, Franz Joseph, on phrenology, 181
Gamma-aminobutyric acid
deciency of, gene therapy for, 426
receptors for, in antinociception, 453454
640
Gonadotroph adenoma
diagnosis of, 4243
endocrinopathy in, 4243
imaging of, 43
medical treatment of, 84
pathology of, 34
H
Hallucinations, thalamocortical dysrhythmia in.
See Thalamocortical dysrhythmia.
Hardy, Jules, transsphenoidal approach of, 810
Headache
in deep brain stimulation, 396
in intraventricular metastasis, 595
in lateral ventricle tumors, 510
in neurocytoma, 483
peripheral nerve stimulation for, 404405
Hemangiopericytoma, parasellar, imaging of,
6870
Hemianopsia, bitemporal, in prolactinoma, 29
Hemiparesis, in lateral ventricle tumor surgery,
523
Hemorrhage, intracranial
in deep brain stimulation, 395
in intraventricular metastasis, 595
in lateral ventricle tumor surgery, 523
in third ventricular tumor surgery, 542
Herniorrhaphy, postoperative pain in, peripheral
nerve stimulation for, 406
Hydrocephalus
after lateral ventricle tumor removal, 523
after third ventricular tumor removal, 542
endoscopic ventriculostomy for, 551552
in choroid plexus tumors, 623626
in Dandy Walker malformation, 613615
in ependymoma, 572, 574
in lateral ventricle tumors, 509
in subependymal giant cell astrocytoma, 584
in subependymoma, 579
in third ventricular tumors, 528
Hydrocortisone, for hypopituitarism, after
pituitary surgery, 129
Hyperalgesia
central mechanisms of, 450451
in gene therapy for pain, 431
Hypercortisolemia, in corticotroph adenoma,
3132, 4548
medical treatment of, 8384
persistent, 141142
Hypernatremia, after pituitary surgery, 124126
Hyperprolactinemia
in acromegaly, 44
in prolactinoma, 2931, 4142
medical treatment of, 8182
persistent, 142143
Hyperthyroidism, in thyrotroph adenoma, 4850
Hypocortisolism, after pituitary surgery, 129130
Hypogonadism
in gonadotroph adenoma, 42
in prolactinoma, 8182
Hyponatremia, after pituitary surgery, 126129
641
Hypothalamus
anatomy of, in third ventricular surgery, 529
astrocytoma of, imaging of, 6566
dysfunction of, after pituitary radiotherapy,
161162
Interhemispheric approaches
to intraventricular meningioma, 565566
to lateral ventricle tumors, 520522
Hypoxanthine-guanine phosphoribosyltransferase
gene transfer, 422423
Immunohistochemistry
in choroid plexus tumors, 470, 623
in ependymoma, 473
in neurocytoma, 485
in subependymoma, 582
642
Intrathecal (continued )
trial of, 355
versus epidural delivery, 354
versus medical treatment, 383
Intraventricular tumors
choroid plexus, 469472, 621631
congenital, 607619
dierential diagnosis of, 469
ependymoma. See Ependymoma.
epidemiology of, 469482. See also specic
tumor types.
glioma, 571591
lateral, 509525
meningioma, 470471, 559569
of third ventricle. See Third ventricular
tumors.
metastatic, 593606
neurocytoma, 470471, 476478, 483508
pathology of, 469482
posterior, 527545
subependymal giant cell astrocytoma, 470471,
475476, 584585
subependymoma. See Subependymoma.
surgical treatment of, endoscopic adjuncts to,
547557
Irrigation, in endoscopic intraventricular surgery,
550551
Lanreotide
for somatotroph adenoma, 83, 140
for thyrotroph adenoma, 84
Lateral ventricles, tumors of, 509525
anatomic considerations in, 510514
blood supply of, 513514
clinical presentations of, 509510
dierential diagnosis of, 510
epidemiology of, 509510
intraoperative considerations in, 514516
outcome of, 522524
postoperative considerations in, 516, 522524
preoperative considerations in, 514
surgical approaches to, 516522
frontal transcortical (middle frontal gyrus),
517518
interhemispheric, 520522
parietal transcortical (superior parietal
lobule), 519520
temporal transcortical (middle temporal
gyrus), 518519
types of, 509
Leksell, Lars, capsulotomy procedure of, 191
Lentiviruses, for gene transfer, for pain
management, 425
Leukotomy
limbic, for psychiatric disorders, 193
depression, 205207, 209, 232
obsessive-compulsive disorders, 205207,
209, 218219, 232
versus cingulotomy, 232
orbitomedial, for obsessive-compulsive
disorder, 218
J
Jacobsen, Carlyle, neurophysiologic studies of,
182
Ki-67 antigen
in choroid plexus tumors, 470
in ependymoma, 473
Knight subcaudate tractotomy, 193
L
Laborit, Henri, psychoactive drug therapy
discovery by, 190
Lactotroph(s), 25. See also Prolactin;
Prolactinoma.
Laminotomy, for spinal cord stimulation implant,
375
Lobotomy
frontal, history of, 182190, 203, 303308
prefrontal, history of, 182190
transorbital, 189
Long Shore and Harbor Workers Act, 349
Lung carcinoma, intraventricular metastasis from,
596597
Luteinizing hormone, 19, 2526
excess of, in gonadotroph adenoma, 34, 4243,
84
Lyerly, James, psychiatric neurosurgical
procedures of, 186187
Lymphocytic adenohypophysitis, imaging in,
7273, 75
Lymphoma, clival, imaging of, 78
corticotroph, 47
for radiotherapy planning, 151152
for stereotactic radiosurgery, 160
gonadotroph, 43
preoperative, 94
prolactinoma, 42
in precentral stimulation, 438
in Rathkes cleft cyst, 63
in schwannoma, 70
in stereotactic cingulotomy, 228, 238
in subependymal giant cell astrocytoma, 584
in subependymoma, 579
in suprasellar aneurysm, 76
in third ventricular tumors, 528
morphometric
in depression, 217
in obsessive-compulsive disorder, 214
643
Magnetoencephalography, in thalamocortical
dysrhythmia, 253256, 260
Mammillary bodies, anatomy of, in endoscopic
surgery, 548549
Mammosomatotroph adenoma, 2728
Manganese superoxide dismutase gene transfer,
for pain management, 430
Mania, transcranial magnetic stimulation for, 292
Mark, Vernon H., on psychiatric neurosurgery,
309310, 315
Mearns, Edward, on psychiatric neurosurgery,
311313
Medical history, in pain management, review of,
340342
Melanotropins, 2021
Melzack-Wall gate theory of pain, 328331, 451
Memory loss, in lateral ventricle tumor surgery,
523
Meningioma
intraventricular, 478, 559569
anatomic considerations in, 559560
clinical presentation of, 560561
incidence of, 559
origin of, 559560
pathology of, 560
644
Meningioma (continued )
radiography in, 561562
treatment of, 561568
observation in, 561, 563
options for, 561, 563
radiosurgery in, 563
radiotherapy in, 563
surgical, 563568
parasellar, imaging of, 6768
Meningitis, in intrathecal medication delivery,
357358
Mental status alterations, in neurocytoma, 483
Metastasis
intraventricular, 478, 593606
assessment of, 599600
classication of, 593
clinical presentation of, 594598
diagnosis of, 594595
histology of, 595598, 602603
incidence of, 593594
location of, 595598
metachronous presentation of, 594595
multiple, 602
outcome of, 604
radiography in, 600602
single, 600602
surgical treatment of, 598604
synchronous presentation of, 594595
to clivus, imaging of, 78
to pituitary, imaging of, 71
N-Methyl-D-aspartate (NMDA) receptors, in
pain transmission, 451453
antagonists of, 360
N
National Commission for the Protection of
Human Subjects of Biomedical and Behavioral
Research, psychiatric neurosurgery and,
312314, 316
Microadenoma, pituitary
imaging of, 59
surgical treatment of, 8992
645
O
Obesity, pain management and, 342
Obsessive-compulsive disorder, 199212
behavioral therapy for, 200201
clinical features of, 200
diagnosis of, SPECT in, 238, 241242
epidemiology of, 200
medical treatment of, 200
neurocircuitry models for, 214216, 218219
neuroimaging in, 214216, 218219
neurosurgery for
capsule anterior limb electrical stimulation,
267274
capsulotomy, 204208, 219, 232233
cingulotomy, 204206, 208209, 216,
225231, 238249
current awareness of, 201202
eectiveness of, 207209
history of, 202203
646
Obsessive-compulsive (continued )
limbic leukotomy, 205207, 209, 218219,
232
modern procedures for, 203205
neuroimaging and neurocircuitry related to,
218219
safety of, 205207
subcaudate tractotomy, 204205, 207, 218,
231232
pathophysiology of, 226
positron emission tomography in, 226,
246248
SPECT in, 237250
diagnostic, 238, 241242
postoperative, 238241, 245249
thalamocortical dysrhythmia in. See
Thalamocortical dysrhythmia.
transcranial magnetic stimulation for, 293294
Occipital headache, peripheral nerve stimulation
for, 377378, 404405
Occipital transtentorial approach
to intraventricular meningioma, 566
to third ventricular tumors, 532, 539541
OCD. See Obsessive-compulsive disorder.
Octreotide
for gonadotroph adenoma, 84
for somatotroph adenoma, 83, 140
for thyrotroph adenoma, 84
intrathecal delivery of, 361
Oligodendroglioma
versus central neurocytoma, 478
versus ependymoma, 472
Oncocytoma, pituitary, pathology of, 35
Opioids. See also Morphine.
endogenous, in antinociception, 453
intrathecal delivery of, 354, 358
Optic chiasm, anatomy of, pituitary surgery and,
17
Optic nerve, anatomy of, pituitary surgery and, 17
Orbitofrontal-subcortical connections
in depression, 216219
in obsessive-compulsive disorder, 214, 218219
interruption of, in subcaudate tractotomy, 193,
204205, 207
Orbitomedial leukotomy, for obsessivecompulsive disorder, neuroimaging in, 218
Orbitozygomatic approach, to parasellar and
suprasellar lesions, 109114
Oxytocin, 2122
P
Pain, chronic, anatomy and physiology of,
445462
antinociception, 452454
central sensitization, 450452
descending systems, 454455
nociception, 445448
peripheral sensitization, 449450
supraspinal plasticity, 455
supraspinal systems, 454454
Pain evaluation, 339352
compensation systems and, 341, 349350
for patient selection, 339340
medical history review in, 340342
physical ndings in, 342343
prior treatment evaluation, 345347
psychologic report review in, 343345
psychosocial issues in, 347348
secondary gain and, 349
Pain management
anatomic and physiologic considerations in,
445462
compliance with, 343, 345
decision not to treat, 340
deep brain stimulation for, 389399
evaluation for, 339352
failure of, 339340, 345
in spinal cord stimulation, 377, 410
gene therapy for, 419435
intrathecal medications for. See Intrathecal
pain management.
neuroaugmentation for
evaluation for, 339352
history of, 327337
psychologic evaluation for, 409417
pain classication for, 329330
peripheral nerve stimulation for. See
Peripheral nerve stimulation.
precentral stimulation for, 334, 437443
regression of benet in, 339340, 345
spinal cord stimulation in. See Spinal cord
stimulation.
terminology of, 329330
Paleospinothalamic tract, in nociception, 447
Palliative care, for psychiatric disorders,
neurosurgery in, 316
Pallidotomy, anterior medial, for thalamocortical
dysrhythmia, 252263
magnetoencephalography with, 253256, 260
patient selection for, 252
results of, 255, 259263
647
carcinoma, 627628
clinical features of, 623624
epidemiology of, 621
hydrocephalus in, 625626
imaging in, 624, 626
outcome of, 628629
pathology of, 621623
treatment of, 624628
Pegvisomant, for acromegaly, 83, 140
648
Pituitary gland
abscess of, imaging in, 71
anatomy of, 5557
dysfunction of, after pituitary radiotherapy,
161162
histiocytosis of, imaging in, 72, 74
histogenesis of, 13
histology of, 2526
hormones of, 1722, 2526. See also specic
hormones.
abnormalities of. See Pituitary tumors,
endocrinopathies in.
hyperplasia of, imaging in, 61
imaging of, 5557. See also Pituitary tumors,
imaging of.
abscess, 71
histiocytosis X, 72, 74
lymphocytic adenohypophysitis, 7273, 75
sarcoidosis, 71
techniques for, 57
tuberculosis, 71
versus hyperplasia, 61
lymphocytic adenohypophysitis of, 7273, 75
sarcoidosis of, imaging in, 71
tuberculosis of, imaging in, 71
tumors of. See Pituitary tumors.
Pituitary tumors
anatomic considerations in, 1123, 5557
embryologic, 1113
gross, 1314
surgical, 1517
vascular, 1415
carcinoma, pathology of, 3637
classication of, 2526
corticotropinsecreting. See Corticotroph
adenoma.
endocrinopathies in, 4154
acromegaly, 4345, 8283, 134, 139141
Cushings disease. See Cushings disease, in
corticotroph adenoma.
gonadotrophin abnormalities, 4243
hyperprolactinemia, 2931, 4142, 8182,
142143
hyperthyroidism, 4850
postoperative management of, 123138
acromegaly, 131, 133
antidiuretic hormone abnormalities,
123129, 132
Cushings disease, 130131, 133134
hypopituitarism, 129130
in nonfunctioning tumors, 132133
in prolactinoma, 131, 134
in somatotroph adenoma, 131, 133
649
650
Pterional approach
to parasellar and suprasellar lesions, 109111
to pituitary tumors, 104
Pumps, for intrathecal pain management,
356359, 383384
Q
Quadrigeminal cistern, anatomy of, in third
ventricular surgery, 531
Quinagolide, for hyperprolactinemia, 8182
R
Rabies-G pseudotyped lentivirus, for gene
transfer, for pain management, 425
Radiation therapy
for choroid plexus carcinoma, 627628
for ependymoma, 576
for intraventricular metastasis, 600602
for meningioma, 563
for neurocytoma, 487491, 493501
for pituitary tumors, 147166
conformal techniques for, 151153
conventional techniques for, 149151
corticotroph adenoma, 142
ecacy of, 147149
external beam, 147153, 161162
in long-term management, 167171
in persistent endocrine activity, 140144
nonfunctioning, 170
planning for, 149
prolactinoma, 143
somatotroph adenoma, 140141, 168
stereotactic. See Stereotactic radiosurgery.
thyrotroph adenoma, 144
toxicity of, 148, 161163
for subependymoma, 583584
Radiosurgery
for intraventricular meningioma, 563
gamma knife
for neurocytoma, 498, 501
for pituitary tumors, 156161
for somatotroph adenoma, 83
linear accelerator-based
for neurocytoma, 501
for pituitary tumors, 155156
proton beam, for pituitary tumors, 154155
stereotactic. See Stereotactic radiosurgery.
Raspberry, William, on psychiatric neurosurgery
regulations, 314
Rathkes cleft cyst, imaging of, 63
651
Sensitization
central, 450452
peripheral, 449450
Sensory disorders
in lateral ventricle tumors, 510
in third ventricular tumor surgery, 542
Septum pellucidum
anatomy of, 511
variants of, 608609
Seroma, in intrathecal medication delivery, 358
Shock therapies, for psychiatric disorders, 184
Sandostatin
for somatotroph adenoma, 83, 140
for thyrotroph adenoma, 144
Sarcoidosis, of pituitary, 71
Somatostatin analogues
for somatotroph adenoma, 83, 140
for thyrotroph adenoma, 84, 144
Schizophrenia
lobotomy for, 308
transcranial magnetic stimulation for, 293
Schwannoma, parasellar, imaging of, 70
Scintigraphy, in somatotroph adenoma, 45
Secondary gain, in pain
chronic, 349
evaluation of, before management, 415
Seizures. See also Epilepsy.
in lateral ventricle tumor surgery, 523
in precentral stimulation, 438, 441
induction of, in transcranial magnetic
stimulation, 294295
Selective serotonin reuptake inhibitors, for
obsessive-compulsive disorder, 200
Sellar tumors, anatomic considerations in,
1516, 57
Somatotroph(s), 25
Somatotroph adenoma
acromegaly in, 4345
after pituitary surgery, 134, 139141
long-term management of, 168169
medical treatment of, 8283
persistent, 139141
densely granulated, 2627
diagnosis of, 45
long-term management of, 168169
medical treatment of, 140, 168
mixed cell, 2729
outcome of, 168169
pathology of, 2629
persistent, 139141
radiotherapy for, 140141
sparsely granulated, 27
652
Somatotroph (continued )
surgical treatment of
persistent, 139140
postoperative endocrine management in,
131, 134
prolactinoma, 9091
proton beam, 154155
somatotroph adenoma, 141
toxicity of, 162163
Stereotactic surgery
cingulotomy. See Cingulotomy.
for pain, 328, 438439
history of, 190193
Spinal cord
compression of, in intrathecal medication
delivery, 358
dorsal horn of, reorganization of, in
sensitization, 451
dorsal root ganglia of, in nociception, 445446
laminar organization of, nociception and,
446447
Somatotroph-lactotroph adenoma, 27
Stereotactic radiosurgery
for intraventricular metastasis, 601602
for neurocytoma, 498501
for pituitary tumors, 153161
gamma knife, 83, 156161
historical view of, 6, 8
linear accelerator-based, 155156
nonfunctioning, 170
653
Synaptophysin
in choroid plexus tumors, 470
in neurocytoma, 485
T
Talairach capsulotomy procedure, 191
Tegmentum, anatomy of, in endoscopic surgery,
549
Tela choroidea, anatomy of, in third ventricular
surgery, 531
Temporal gyrus, middle, approach to, to
intraventricular meningioma, 564565
Temporal horn, anatomy of, 512
Temporal transcortical (middle temporal gyrus)
approach, to lateral ventricle tumors, 518519
Tetracycline, for gene transfer, for pain
management, 422
Thalamocortical dysrhythmia, 251265
pathophysiology of, 251, 259, 262
surgery for, 252263
magnetoencephalography with, 253256,
260
patient selection for, 252
results of, 255, 259263
strategy for, 252253, 259
Thalamostriate vein, anatomy of, 512513
Thalamotomy
central lateral, for thalamocortical
dysrhythmia, 252263
654
Thyrotroph (continued )
pathology of, 3233
radiotherapy for, 144
surgical treatment of
persistent, 143
postoperative endocrine management in,
131132, 134
preoperative management in, 94
Tics, transcranial magnetic stimulation for, 293
Tourette syndrome
neuroimaging and neurocircuitry in, 214
transcranial magnetic stimulation for, 293
Tractotomy, subcaudate, for psychiatric
disorders, 193
depression, 204205, 207, 218
obsessive-compulsive disorder, 204205, 207,
218, 231232
versus cingulotomy, 231232
Transcallosal approaches
to lateral ventricle tumors
anterior, 520521
posterior, 521522
to third ventricular tumors, 532, 534, 538539
Transcortical approaches, to lateral ventricle
tumors, 517520
Transcranial approaches
to parasellar and suprasellar lesions, 109122
to pituitary tumors, 45, 9394, 103104
Transcranial magnetic stimulation, 283301
animal models of, 287
drug use with, 293
electrical requirements of, 285
for anxiety disorders, 293294
for depression, 288293
maintenance, 292
meta-analysis of, 289292
with intentional seizure generation, 294295
for eloquent cortex localization, 287
for epilepsy, 286287, 293
for mania, 292
for movement disorders, 293
for schizophrenia, 293
functional imaging with, 287288
high-frequency, 285286
mechanisms of action of, 284286
paired-pulse, 286287
repetitive, 284
research uses of, 286288
safety of, 286
versus electroconvulsive therapy, 287289,
291292, 294
U
Ultrasonography, in lateral ventricle tumors, 514
Urinary free cortisol test, in corticotroph
adenoma, 46
V
Vagus nerve, electrical stimulation of
clinical applications of, 280
for depression, 275282
anatomic considerations in, 275
clinical trials of, 276
long-term results of, 278279
mechanism of action of, 277, 279280
open-label study of, 277278
placebo-controlled study of, 278279
practical considerations in, 280281
preclinical investigations of, 275276
Visual disorders
after pituitary radiotherapy, 162163
in lateral ventricle tumor surgery, 52
in neurocytoma, 483
in prolactinoma, 29
in third ventricular tumor surgery, 542
655