International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 156158

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International Journal of Pediatric Otorhinolaryngology

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Case report

Pharyngeal tonsil manifestation of Langerhans cells sarcoma: A case report

and review of the literature
Ba Luo a,b, Bian Pian b, Zhou Peng a, Du Jintao a, Liu Shixi a,*
a
b

Department of Otorhinolaryngology-Head and Neck Surgery of West China Hospital of Sichuan University, 37# Guoxuexiang, Chengdou, Sichuan, China
Department of Otorhinolaryngology of Peoples Hospital of The Tibet Autonomous Region, 18# Linkor Northern Road, Lhasa, China

A R T I C L E I N F O

A B S T R A C T

Article history:
Received 1 December 2009
Received in revised form 20 June 2010
Accepted 23 June 2010
Available online 21 July 2010

Langerhans cell sarcoma (LCS) is an extremely rare malignant disease with multi-organ involvement and
a poor prognosis. LCS involvement of tonsil has not been previously reported. However, we present
herein a 10-year-old Tibetan boy distressed by LCS arising on the pharyngeal tonsil. Owing to local
traditions and customs, he endured a long period of treatment with traditional Tibetan medicine which
provided only slight relief at best that delayed an accurate diagnosis and scientic medical treatment.
Subsequently, based on CT and MRI scans, combined with pathology features positive immunophenotype for the CD1a and S-100 proteins conrmed the diagnosed of LCS. We performed a surgical
resection along with a regimen of E-CHOP chemotherapy was prescribed as new protocols. As a result the
patient complete remission symptoms and without relapse has been 20 months.
2010 Elsevier Ireland Ltd. All rights reserved.

Keywords:
Langerhans cell sarcoma
Pharyngeal tonsil
Immunohistochemical
E-CHOP

1. Introduction
Langerhans cell sarcoma (LCS) is a neoplastic proliferation of
Langerhans cells that have overtly malignant cytologic features
with an aggressive spread. LCS is a very rare disease and
theoretically shows multi-organ involvement, including the skin,
lymph nodes, lung and bone. Patients have a poor prognosis and a
small chance of survival, despite conventional combination
chemotherapy, surgery, and radiotherapy [1]. LCS involvement
of the tonsil has not been previously reported, and there is a lack of
knowledge regarding the prognosis and therapeutic guidelines.
This report describes the medical history and histology with
immunohistochemical features of the case. An E-CHOP regimen as
a new chemotherapeutic protocol was administered post-operatively. A brief review of the relevant literature that might
contribute to recognition and management of LCS is provided.
2. Case report
A 10-year-old Tibetan boy was taken to the Traditional Tibetan
Medicine Hospital of the Tibetan Autonomous Region (China) for
evaluation of a sore throat, dysphagia to solid foods, and an
intermittent, unexplained low-grade fever for approximately 5
months. Upon physical examination, a 2.5 cm  3.2 cm painful,
glossy, nodular mass was noted on the right pharynx spreading to

* Corresponding author. Tel.: +86 02885422436.

E-mail address: basangnorpu88@sina.com.cn (L. Shixi).
1871-4048/$ see front matter 2010 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.pedex.2010.06.004

the tonsil. A neck examination revealed no enlarged lymph nodes.

He received traditional Tibetan medicine treatment for >4 months.
He experienced little relief and the mass did not shrink in size.
He was admitted to a local Western-style hospital that performed
a computed tomography (CT) scan which revealed a 4 cm 
4 cm  3.5 cm middle-density tumor on the right pharynx (Fig. 1).
The pathologic examination revealed an inammatory reaction,
which did not respond to antibiotics. Finally, the patient was sent
to the West China Hospital for further diagnosis and treatment. A
magnetic resonance image (MRI) revealed abnormal signals in the
right pharyngeal area and locally invading the parapharyngeal
space with a few lymph nodes in the sub-maxillary space (Fig. 1). A
surgical resection was performed. The partial neoplastic tissue was
sent for frozen section. The pathologic results revealed malignant
cytologic features, but a denitive diagnosis was not rendered.
Intra-operatively, the right tonsil was inltrated with tumor and
without distinct borders. Extensive surgical resection with leaving
a 1 cm free margin from the pharyngeal mucosa, as well as a total
right tonsillectomy. Histologically, the tumor cells showed diffuse
inltrative growth into the tonsillar fossa and characterized by a
proliferation of enlarged, round cells with folded and indented
nuclei (Fig. 2B). The nal immunohistochemical results revealed
that the tumor cells were positive for anti-CD1a and S-100
antibodies (Fig. 2A and C). Consequently, the diagnosis of LCS was
made. Under the care of an oncologist, he was began E-CHOP
chemotherapy regimen (etoposide, 70 mg on d13; vincristine,
1.6 mg on d1 and 8; cytarabine, 0.85 mg on d1; adriamycin, 45 mg
on d1; and prednisolone, 60 mg on d114.) After two cycles, he was
leukopenic. The laboratory data on presentation were as follows:

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157

Fig. 1. MRI and CT scan revealed a mass in the fundus of the right side pharyngeal area and part of parapharyngeal space.

white blood cell count (WBC), 2.1  109/L (stabs, 5%; segmenteds,
65%; lymphocytes, 19%; monocytes, 5.6%; eosinophils, 3.2%; and
basophils, 1.2%). Following a berbamine infusion, to the WBC count
returned to normal. The oncologist suggested low-dose radiation
therapy for the resected primary lesion, but the boys parents
declined. We continue to follow-up the boy and there has been no
recurrent regional or distant metastasis almost 2 years.
3. Discussion
Langerhans cell tumors are neoplasms currently classied by
the World Health Organization as Langerhans cell histiocytosis
(LCH) and LCS. LCH is a proliferating disorder of Langerhans
cells, whereas LCS is a neoplastic proliferation of Langerhans
cells that have malignant cytologic features [2]. The diagnosis of
LCS relies on malignant cytologic features and a combined
typical immunophenotype with consistent expression of CD1a
and S-100 proteins, as well as Langerin (CD207). It also has the
appearance of a typical Birbeck granule [3]. LCS can occur at any
age and occurs more commonly in females. LCS may also involve
multiple systems or tissues, such as bone, lung, brain, skin/
mucous membranes, lymph nodes, liver, and various other soft
tissues. It has rapidly growing and aggressive clinical behavior.
Prior to the widespread use of immunohistochemistry studies,
many of these tumors were diagnosed as non-Hodgkins
lymphoma or other malignant tumors [4]. Currently, the

availability of reliable cell markers, this kind of neoplasm has

become more readily recognizable and accurately diagnosed [5].
However, according to Ben-Ezra et al. [6] LCSs are often not
easily recognized morphologically as a Langerhans cell derivative, but are diagnosed based on CD1a staining. As well as in a
recent study, Verdijk et al. [7] reported the lack of Birbeck
granules in Langerhans cells to be associated with a mutation in
the Langerin gene. The Langerin gene represents a very specic
marker for Langerhans cells and derived tumors, even in Birbeck
granules-negative cases [8].
In the present case, local conventional practices dictated that
the patient rely on traditional Tibetan medicine whether or not
his symptoms were relieved, and facilities for cell markers and
immunohistochemistry were unavailable in the hospital in
Tibet. Thus, there was a failure to promptly treat the boy at rst.
Nevertheless, in West China hospital although we did not
demonstrate the ultrastructural features, but the histomorphologic and immunohistochemical ndings supported the diagnosis of LCS. The knowledge of prognosis and therapeutic
guidelines were lacking in rarity of this kind of tumor, thus
we should provide a new proposal for the therapeutic plan. A
PUBMED search of the years from 1960 to 2010 revealed 14
relevant reports of LCS, but none of the reports included LCS
involving the pharynx, shows a poor prognosis and a short
survival period, despite conventional combination chemotherapy, surgery, and radiotherapy [9]. Thus, still an optimal

Figure 2. (A) IHC staining positivity anti-CD1a; (B) HE staining tumor cells nuclei are grooved, indented, folded or lobulated; and (C) IHC strong positivity anti-S100.

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B. Luo et al. / International Journal of Pediatric Otorhinolaryngology Extra 6 (2011) 156158

treatment strategy or neoadjuvant for LCS must be considered to

be under investigation. Chemotherapy is commonly undertaken
using CHOP (cyclophosphamide, prednisolone, adriamycin, and
vincristine) regimen in a standard dose adapted to the common
lymphoma treatment protocols [10]. Whereas we provided
thoroughly resected the tumor with wide margins and
prescribed the E-CHOP regimen chemotherapy as new protocols
in the present case. Fortunately, its seems to be an effective
therapy. After surgical resection and two subsequent cycles of
chemotherapy, the patient showed no apparent recurrent or
metastatic signs >20 months later, without any other adjuvant
therapy. Of course the tumor size appears to be an important
prognostic factor [11]. In our patient, the tumor was localized
with no clinical evidence of distant lymphadenopathy or other
organ involvement thus the prognosis was favorable. Although
our treatment protocol seems to benet patients with LCS, but it
still needs longer follow-up, as well as more patients treated to
conrm the therapeutic effects.
In conclusion, we have reported an unusual case of primary
pharyngeal LCS, arising on the right pharyngeal tonsil of 10year-old child. This case shows the necessity of careful attention
to clinical symptoms as well as diagnostic pathology procedures
in such a rare case. We treated the patient with E-CHOP in a
standard dose for new treatment protocols. Although the postoperative follow-up has been >20 months and not recurrent
evidence. However, there are no large studies addressing ECHOP for this neoplasm. Therefore further reports are needed to
conrm its therapeutic effect. So a careful long-term follow-up
is mandatory.
Conict of interest
Authors declared no conict of interest.

Acknowledgements
The patient and his family were informed that data from the
case would be submitted anonymously for publication, and they
gave their consent.
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