Benign Esophageal Tumors and Cysts

Benign tumors of the esophagus are rare, constituting only 0.5 to 0.8% of all esophageal
neoplasms.
Approximately 60% of benign esophageal neoplasms are leiomyomas, 20% are
cysts, and 5% are polyps.
[129]

Leiomyomas

Leiomyomas are the most common benign tumors of the esophagus. These intramural tumors
typically occur between 20 and 50 years of age, have no clear-cut gender preponderance, and are
multiple in 3 to 10% of patients. More than 80% of these tumors occur in the middle and lower
thirds of the esophagus, rarely in the cervical region. Histologically, the tumors consist of
interlacing bundles of smooth muscle cells with or without calcification. These tumors do not
infiltrate surrounding tissue, so the overlying mucosa is rarely, if ever, invaded.
DIAGNOSIS

Despite infringement into the lumen by the tumor, symptoms of dysphagia and vague retrosternal
pressure or pain are produced only by large tumors (larger than 5 cm) because of the
distensibility of the uninvolved esophagus. Most are found incidentally at autopsy and are
asymptomatic. Esophageal symptoms prompt performance of a barium swallow and/or an
endoscopic examination. The barium swallow appearance is distinctive because the welllocalized mass has a smooth surface and distinct margins, and it is not circumferential. Most
frequently, a leiomyoma is seen on a chest x-ray as a posterior mediastinal mass or is found
unexpectedly during endoscopic examination. During endoscopy, the mucosa is intact, and the
extrinsic mass narrows the lumen but can easily be displaced and passed with the
esophagoscope. Endoscopic biopsy should be avoided because adherence of the tumor to the
mucosal biopsy site may complicate subsequent surgical resection. Esophageal ultrasonography
confirms the diagnosis of leiomyoma, which appears as a hypoechogenic homogenous area
beneath intact mucosa.
TREATMENT

As a general rule, excision of symptomatic leiomyomas or those larger than 5 cm is advised.

Asymptomatic or smaller tumors discovered incidentally can be observed and followed.
Although excision of the esophageal tumor provides the only absolute proof that it is benign,
leiomyomas have such a characteristic radiographic appearance, generally slow growth rate, and
low risk of malignant degeneration that periodic follow-up of these lesions is reasonable. The
malignant variant of leiomyosarcoma is extremely rare.
Polyps of the cervical esophagus (20% of benign tumors) are intraluminal lesions that may cause
dysphagia or may even be regurgitated into the larynx with the potential for asphyxiation. They
are composed of a fibroelastic core and usually are covered with normal epithelium. The
preferred approach for resection is through a lateral cervical esophagomyotomy, thereby
delivering the polyp and resecting the mucosal origin of the pedicle under direct vision.
Esophageal polyps have also been removed endoscopically by electrocoagulating the pedicle.
Lipomas, vascular tumors, and neurofibromas are extremely rare, but they must be removed to
control symptoms or to exclude malignancy.
When resection is indicated, benign tumors of the middle third of the esophagus are approached
through a right thoracotomy; those in the distal third are approached through a left thoracotomy.
The tumor is located, and the overlying longitudinal esophageal muscle is split in the direction of
its fibers, to reveal the mass. The tumor is then gently dissected away from contiguous tissues

and the underlying submucosa. Once the tumor has been enucleated, the longitudinal muscle
should be reapproximated if possible. The operative technique for excision of an esophageal cyst
is identical to that for a benign tumor. Esophageal resection may be required for either giant
leiomyomas of the cardia that involve the adjacent stomach or for diffuse esophageal
leiomyomatosis, although multiple enucleations may be performed if possible.
[83]

Using minimally invasive surgical techniques, the surgeon's approach from the left side of the
patient's chest allows access to the distal esophagus and performance of a concomitant antireflux
procedure if necessary. The right side of the chest is preferable for access to lesions of the middle
and upper esophagus, in which the aorta interferes with left-sided approaches.
TABLE 37-6 -- Classification of Benign Esophageal Tumors
I. Epithelial tumors
A. Papillomas
B. Polyps
C. Adenomas
D. Cysts
II. Nonepithelial tumors
A. Myomas
1. Leiomyomas
2. Fibromyomas
3. Lipomyomas
4. Fibromas
B. Vascular tumors
1. Hemangiomas
2. Lymphangiomas
C. Mesenchymal and other tumors
1. Reticuloendothelial tumors
2. Lipomas
3. Myxofibromas
4. Giant cell tumors
5. Neurofibromas
6. Osteochondromas
III. Heterotopic tumors
A. Gastric mucosal tumors
B. Melanoblastic tumors
C. Sebaceous gland tumors
D. Granular cell myoblastomas

E. Pancreatic gland tumors

F. Thyroid nodules

Invasion of or attachment to the muscle or mucosa is rare, so the tumor can be easily separated.
Electrocautery should not be used for fear of injuring the mucosa. A small muscle defect can be
covered by suturing the muscle layers in a transverse orientation. If the muscle gap is large, an
onlay patch of parietal pleura is an option.
The operative technique for excision of an esophageal cyst is identical to that for a benign tumor.
However, cysts occasionally communicate with the esophageal lumen or adhere to the mucosa
because of prior cyst infection. In these instances, it is prudent to unroof the cyst and to cauterize
its mucosal lining rather than to risk extensive damage by attempting to dissect the cyst free of
all attachments.
[83]

Esophageal Cancer
Despite advances in surgery, critical care, radiotherapy, and chemotherapy, esophageal cancer
afflicts some 13,000 new patients in the United States each year, and almost matching that figure
is the expected death rate of 12,000 patients. The disease represents 4% of newly diagnosed
cancers. major shift in the histologic type of tumors has occurred. Traditionally, esophageal
cancer has been squamous cell in patients with the usual risk factors for other aerodigestive tract
carcinomas, specifically smoking (5-fold) and alcohol (5-fold) abuse. Heavy smoking and heavy
drinking combine to increase the risk 25- to 100-fold. Remarkably, within North America and
Europe, the incidence of adenocarcinoma rose 100% in the 1990s, and it had a strong correlation
with reflux, Barrett's metaplasia, and dietary factors (e.g., fat).
]

[78]

Although the origin of this shift remains unknown, carcinoma of the esophagus now appears to
affect younger, healthier patients. Nutritional factors and potential carcinogens have been
incriminated, including alcohol, tobacco, zinc, nitrosamines, malnutrition, vitamin deficiencies,
anemia, poor oral hygiene and dental caries, previous gastric surgery, and long-term ingestion of
hot foods or beverages. An increased incidence of esophageal carcinoma is noted in patients with
familial keratosis. Some esophageal lesions are premalignant, including achalasia, reflux
esophagitis, Barrett's (columnar epithelial-lined) esophagus,
radiation esophagitis,
caustic
burns, Plummer-Vinson syndrome, leukoplakia, esophageal diverticula, and ectopic gastric
mucosa. The rising prevalence of adenocarcinoma associated with Barrett esophagus suggests a
possible link to untreated or silent gastroesophageal reflux. This observation potentially defines
high-risk patients.
[142]

[145]

Esophageal cancer is notorious for its aggressive biologic behavior; it infiltrates locally, involves
adjacent lymph nodes (Figs. 37-28 and 37-29) , and metastasizes widely by hematogenous
spread. Lack of an esophageal serosal layer tends to favor local tumor extension. Tumors of the
upper and middle thirds infiltrate the tracheobronchial tree, aorta, and left recurrent laryngeal
nerve, whereas lower-third tumors may invade the diaphragm, pericardium, or stomach. The
extensive mediastinal lymphatic drainage, which communicates with cervical and abdominal
collateral vessels, is responsible for the finding of mediastinal, supraclavicular, or celiac lymph
node metastasis in at
TABLE 37-7 -- Esophageal Cancer Risk Factors

CONSUMPTION OF
Tobacco
Alcohol
UNDERCONSUMPTION OF
Fruits
Fresh meat
Riboflavin
Beta-carotene
Vitamin C
Magnesium
Vegetables
Fresh fish
Niacin
Vitamin A
Vitamin B complex
Zinc
PREDISPOSING CONDITIONS
Tylosis
Caustic injury
Esophageal webs
Achalasia
Barrett's esophagus
Esophageal diverticula
OTHER EXPOSURE
Asbestos
Ionizing radiation
Exceptionally hot beverages (tea)
Location: Middle East, South Africa, northern China, southern Russia, India
least 75% of patients with esophageal carcinoma. Cervical esophageal cancers drain to the deep
cervical, paraesophageal, posterior mediastinal, and tracheobronchial lymph nodes. Lower
esophageal tumors spread to paraesophageal, celiac, and splenic hilar lymph nodes. Distant
spread to liver and lungs is common. The prognosis for patients with invasive squamous cell
carcinoma is poor; the overall 5-year survival for patients with treated tumors is 5 to 12%.
Extraesophageal tumor extension is present in 70% of cases at the time of diagnosis, and the 5year survival is only 3% when lymph node metastases are present, compared with 42% when no
lymph node spread has occurred.

Histologically, approximately 95% of esophageal cancers worldwide are squamous cell

carcinomas. Early forms of esophageal cancer have been variously termed carcinoma in situ,
superficial spreading carcinoma, and intramucosal carcinoma. They constitute fewer than 5% of
all resected cases, are asymptomatic, and may take 3 to 4 years to progress to invasive squamous
cell carcinoma. Endoscopically, carcinoma in situ most often presents as a slightly raised,
granular, reddish, plaquelike lesion.
Squamous cell carcinoma arises from the mucosa of the esophagus. Histologically, it is
characterized by invasive sheets of cells that run together and are polygonal, oval, or spindleshaped. Located mainly in the thoracic esophagus, approximately 60% of these tumors are found
in the middle third and about 30% in the distal third. Squamous cell neoplasms have four major
gross pathologic presentations. (1) fungating: predominantly intraluminal growth with surface
ulceration and extreme friability that frequently invades mediastinal structures; (2) ulcerating:
flat-based ulcer with slightly raised edges; hemorragic, friable with surrounding induration; (3)
infiltrating: a dense, firm, logitudinal and circumferential intramural growth pattern; and (4)
polypoid: intraluminal polypoid growth with a smooth surface on a narrow stalk (fewer than 5%
of cases). A 5-year survival of 70% is associated with the polypoid tumor compared with a
less than 15% 5-year survival for all other types.
[130]

[143]

Adenocarcinoma is now the most common cell type of esophageal cancer. Adenocarcinoma
arises from the superficial and deep glands of the esophagus, mainly in the lower third of the
esophagus, especially near the gastroesophageal junction. Men have an eightfold higher risk than
women. Esophageal adenocarcinoma may have one of three origins: (1) malignant degeneration
of metaplastic columnar epithelium (Barrett's mucosa), (2) heterotopic islands of columnar
epithelium, or (3) the esophageal submucosal glands. Gastric adenocarcinoma may also involve
the esophagus secondarily. Unlike the mucin-secreting cells of origin, adenocarcinoma
cytologically has a reduced cytoplasmic-nuclear ratio. A loss of cellular polarity demonstrates
variable atypia and nuclear size, enlarged nucleoli, and increased mitoses. Gastroesophageal
junction tumors arise initially as flat or raised patches of mucosa. They may subsequently
ulcerate and become large (up to 5 cm) nodular masses. Tumor size is related to prognosis. For
tumors smaller than 5 cm, 40% are localized, 25% have spread beyond the esophagus, and 35%
have metastasized or are unresectable. For tumors that are more than 5 cm in length, 10% are
localized, 15% have invaded mediastinal structures, and 75% have metastasized.
[152]

As with squamous cell carcinoma, adenocarcinoma of the esophagus exhibits aggressive

behavior with frequent transmural invasion and lymphatic spread. Because many of these tumors
arise in the distal esophagus, spread to paraesophageal, celiac axis, and splenic hilum lymph
nodes is common. Metastases to the lung and liver are frequent. The 5-year survival of patients
with esophageal adenocarcinoma is only 0 to 7%, with the presence of lymph node metastases
exerting a significant negative effect on survival.
Patients with chronic reflux disease develop Barrett esophagus (metaplastic columnar epithelium
replaces the distal squamous mucosa thought to be attributable to prolonged exposure of the
distal esophageal mucosa to gastroesophageal reflux). Barrett's esophagus is of clinical
importance because adenocarcinoma occurs in patients with Barrett's esophagus at a rate 30 to 40
times greater than that of the general population. Although the true incidence of Barrett's
esophagus in the general population is unknown, adenocarcinoma arises in approximately 8 to
15% of patients with a columnar-lined esophagus. The finding of dysplasia in Barrett's mucosa
is a prognostic sign of impending malignant degeneration,
with severe dysplasia virtually
synonymous with carcinoma in situ and an indication for resection.
[142]

[136]

Diagnosis

Symptoms of esophageal carcinoma may be insidious, beginning as nonspecific retrosternal

discomfort or indigestion, followed by the common symptoms of dysphagia and weight loss.
Because of the elasticity of the esophagus, two thirds of the lumen must be obstructed to produce
dysphagia. Patients complain of
TABLE 37-8 -- Symptoms of Esophageal Cancer
Symptom

Patients With Symptom (% )

Dysphagia

87-95

Weight loss

42-71

Vomiting or regurgitation

29-45

Pain

20-46

Cough or hoarseness

7-26

Dyspnea

food "getting stuck," often directly at the location of the lesion. Pain can be caused by spasm or
contractions proximal to an obstruction, tumor invasion, or interference with swallowing, or it
may be related to metastases into the surrounding esophageal lymph nodes. Less frequent
symptoms are coughing or hoarseness associated with tumors of the cervical esophagus. As the
tumor enlarges, esophageal obstruction results in progressive weight loss, regurgitation, and
pulmonary damage, which, together with tobacco and alcohol abuse, result in poor general
physical condition. All these factors have a negative impact on morbidity and mortality.
Because dysphagia is the presenting complaint in 80 to 90% of patients with esophageal
carcinoma, any adult who complains of progressive dysphagia warrants esophagoscopy to rule
out carcinoma. Likewise, esophagoscopy and biopsy are mandatory in every patient with an
esophageal stenosis. The tumor may be advanced sufficiently to be identified on a chest x-ray as
an abnormal azygoesophageal recess, widening of the mediastinum, or posterior tracheal
indentation. A barium swallow will show the extent of the tumor and location, if the tumor
distorts the esophageal lumen, and the presence of obstruction or fistulas. The CT or endoscopic
ultrasound examination can determine the anatomic location and enlargement of the mediastinal,
perigastric, or celiac lymph nodes. Esophagoscopy is required to diagnose and determine the
extent of longitudinal intramural tumor spread. The entire esophagus is visualized, and brush
cytology plus biopsy tissue samples may be obtained for histologic analysis. The accuracy of
brush cytology alone is about 85 to 97%, and that of biopsy alone ranges from 83 to 90%. The
accuracy of the combination of brush cytology and biopsy is more than 97%. If the lesion
remains undiagnosed by biopsy or brush cytology because of the depth of the tumor, endoscopic
ultrasound-guided fine-needle aspiration will further increase the diagnostic yield. Unfortunately,
programs for early detection of esophageal carcinoma using mass screening of patients with
barium esophagograms, flexible fiberoptic esophagoscopy, and exfoliative cytology.
[42]

[96]

Staging

Once the diagnosis of esophageal carcinoma has been histologically established after
esophagoscopy and biopsy, staging of the tumor is the next critical step in determining which
therapeutic option is appropriate (Fig. 37-31) . The stage of a tumor is classified most frequently
by the staging system devised by the American Joint Committee on Cancer. This system is a
TNM-based system. The "T" (tumor) indicates the progressive degree (1 to 4) of invasion of the

tumor into the esophageal wall. "N" stands for nodal involvement, and "M" represents distant
metastasis (Table 37-9) .
Prognosis and outcomes are determined by stage. Five-year survivals for esophageal cancer are
as follows: Stage I, 50 to 55%; Stage II, 15 to 38%; Stage III, 6 to 17%; and Stage IV less than
5% (Table 37-10) .
[36]

[33]

LYMPH NODES (N STAGE)

Until recently, surgical exploration with lymph node sampling has been the standard means for
definitively staging esophageal cancer patients. Advances in imaging technology have greatly
changed the preoperative staging schema. Lymph node involvement may be assessed by
endoscopic ultrasound, CT, positron emission tomography (PET), or video-assisted thoracoscopy
and laparoscopy (Table 37-11) .
Endoscopic ultrasound can assess the size, shape, border, and internal echo characteristics of the
lymph node. CT and endoscopic ultrasound imaging alone rely on the anatomic size of the node
as a predictor of malignancy, but they cannot differentiate between hyperplastic nodes and nodes
enlarged because of metastasis. Endoscopic ultrasound and CT can then be used for imagedirected fine-needle aspiration of mediastinal or celiac nodes. Endoscopic ultrasound has the
advantage of "real-time" imaging during fine-needle aspiration.
Histologic examination of lymph nodes is currently standard for evaluating N stage disease, yet
patients with negative nodal involvement often have recurrent disease. To decrease this error,
immunohistochemical analysis has been evaluated to predict tumor occurrence in patients with
negative nodal involvement by histologic criteria. Monoclonal anti-epithelial cell antibody BerEP4 lymph node staining may be an independent prognostic indicator in esophageal cancer,
indicative of advanced tumors at the time of staging, rather than an indicator of sites of
subsequent
TABLE 37-9 -- Stage Grouping of Esophageal Cancer
Stage 0

T0N0
T is N 0 M0

Stage I

T 1 N 0 M0

Stage II

IIA T 2 N0 M 0
T 3 N 0 M0
IIB T 1 N 1 M0
T 2 N 1 M0

Stage III

T 3 N 1 M0
T 4 any N M 0

Stage IV
T: PRIMARY TUMOR
T 0 No evidence of a primary tumor
T is Carcinoma in situ (high-grade dysplasia)

any T any N M 1

T 1 Tumor invading the lamina propria, muscularis mucosae, or submucosa but not breaching the
boundary between submucosa and muscularis propria
T 2 Tumor invading muscularis propria but not breaching the boundary between muscularis
propria and periesophageal tissue
T 3 Tumor invading periesophageal tissue but not adjacent structures
T 4 Tumor invading adjacent structures
N: REGIONAL LYMPH NODES
N 0 No regional lymph node metastasis
N 1 Regional lymph node metastasis
M: DISTANT METASTASIS
M 0 No distant metastasis
M 1 Distant metastasis
relapse. Video-assisted thoracoscopy and laparoscopy have been found to be accurate, although
invasive, lymph node staging techniques (around 90 to 94%).
]

DISTANT METASTASIS (M STAGE)

Endoscopic ultrasound is especially suited to visualize lymph nodes around the celiac axis and
the left liver lobe (both considered distant metastases). CT is specific for liver, lung, and pleural
metastases larger than 2 cm in diameter,
but evaluation with fine-needle aspiration or
transbronchial biopsy is necessary for the determination of malignancy. Bronchoscopy is
required for patients with tumors of the upper and middle third of the esophagus to view the
pharynx, larynx, and tracheobronchial tree for synchronous and metachronous malignancies. If a
patient complains of bone pain, a bone scan should be performed.
[165]

[57]

The plain chest x-ray is abnormal in only 50% of patients with esophageal cancer, with findings
such as an air-fluid level in the obstructed esophagus in the posterior mediastinum, a dilated
esophagus, abnormal mediastinal soft tissue representing adenopathy, a pleural effusion, or
pulmonary metastasis being most common. The chest film, however, may be deceivingly normal
even in patients with advanced disease.
COMPUTED TOMOGRAPHY

CT scanning of the chest and upper abdomen is standard and permits evaluation of the
esophageal wall thickness (should normally not exceed 5 mm), assessment of direct mediastinal
invasion by the tumor, and the presence of regional lymphadenopathy and/or pulmonary, liver,
adrenal, and distant nodal metastases. CT enables one to accurately detect distant metastases (M
stage), especially in the liver,
and to evaluate adjacent organ (T 4 ) invasion, especially
tracheobronchial.
Regional adenopathy that can be resected immediately adjacent to the
esophagus does not preclude esophagectomy. However, histologically documented distant
metastatic (Stage IV) esophageal carcinoma (e.g., liver, pulmonary, or supraclavicular lymph
node) contraindicates esophagectomy, because the expected survival is only 6 to 12 months.
[73]

[133]

[133]

Although CT criteria of invasion of mediastinal structures have been well described in the
literature, the only absolute confirmation of unresectability is fine-needle aspiration histology or
operative exploration. The accuracy of CT is too low for definitive staging. The accuracy for N

stage ranges from 39 to 74% and from 33 to 57% for T staging alone.
Many esophageal
carcinomas deemed unresectable on the basis of a CT scan are found to be resectable at surgery
because of the proximity of the esophagus to several vital mediastinal structures.
[155] [163]

[131]

ENDOSCOPIC ULTRASOUND

Endoscopic ultrasound is the method of choice to determine depth of tumor invasion and
regional nodal disease and involvement of adjacent structures, with an overall accuracy to 92%.
For patients in whom the probe can be positioned within the esophageal lumen involved by
tumor, endoscopic ultrasound has an 86% accuracy in defining involved mediastinal lymph
nodes. Impassible stenosis has been reported in 25 to 43% of patients with cancer.
[100]

A significant error associated with endoscopic ultrasound T staging is to overstage 7 to 11% of

early disease. [ ] [ ] Diagnostic accuracy ranges from 84 to 92%, with increasing accuracy
directly correlated with increasing T stage [ ] [ ] (about 85% accuracy for T 1 to more than 95%
for T 4 disease). A learning curve is involved with using endoscopic ultrasound to stage
esophageal tumors. [ ]
146

158

146

161

134

T 1 and T 2 masses are located within the esophageal wall, whereas T 3 and T 4 masses invade the
muscularis (extraesophageal). Because the muscularis propria is only millimeters thick, staging
errors can result in misclassification of the tumor (intraesophageal versus extraesophageal) and
may lead to errors in treatment choice. Irregular tumor border and interrupted muscularis propria
( subjective criteria to discriminate T 2 and T 3 disease) are inaccurate; however, overall mass
thickness and extraluminal mass thickness ( objective measurements of mass thickness) are
highly accurate. Accurately detecting T 4 disease recognizes patients with advanced disease and
precludes them from unnecessary surgical intervention while reducing associated morbidity and
costs. The Japan Esophageal Oncology Group
has assessed the accuracy of preoperative
staging of resectable esophageal cancer, using esophagography, esophagoscopy, and
percutaneous and endoscopic ultrasonography. Overall, the accuracy of stage grouping was 56%.
[15

[105]

Endoscopic ultrasound tends to understage lymph nodes, with an accuracy ranging from 50 to
88% for N stage.
Sensitivity decreases as distance increases from the esophageal wall. The
rising incidence in distal and gastroesophageal junction adenocarcinoma increases the need for
accurately staging celiac lymph nodes.
The sensitivity and specificity of endoscopic
ultrasound for determining the presence or absence of malignant celiac nodes are 72 and 97%,
respectively, with a decrease in sensitivity for nodal metastases smaller than 1 cm in diameter.
Advances in endoscopically guided fine-needle aspiration allow direct cytologic evaluation of
lymph nodes or masses within 5 cm of the esophagus (Fig. 37-33).
[163]

[133]

Ultrasonic miniprobes enable safe passage through high-grade malignant strictures and achieve
both higher accuracy rates for T staging and similar rates for N staging.
[87]

POSITRON EMISSION TOMOGRAPHY

The PET scan is a more expensive technique that appears to increase the recognition of distant
metastasis. The main advantage of PET is that it does not rely on anatomic or structural
distortion for detecting malignancy. PET produces whole-body images in three dimensions, but
without anatomic resolution, to assess both metastatic spread and primary disease. The best
technology, although not widely available, for detection of distant metastases is PET. PET is 88%
sensitive, 93% specific, and 71 to 91% accurate for identifying distant metastasis.
Overall,
PET can identify distant metastases and results in upstaging of approximately 20% of patients
who have no distant metastases detected with conventional staging. The accuracy, sensitivity,
[86]

[44] [86]

[80]

and specificity of PET for detecting distant metastases are 90% or better.
accuracy in detecting nodal involvement.

[86]

PET has a 48 to 76%

The disadvantages of PET scanning : PET is a low-resolution functional technique based on

increased cellular metabolism and not on anatomic changes, the level of tumor invasion (T stage)
is not reliably predicted. Although PET scan is more reliable than CT alone for identifying
metastatic disease, the functional advantages of PET and the structural advantages of CT
combine to enhance the detection rate for metastasis to 80 to 90% accuracy. If the tumor is
anatomically evident, but metabolically inactive, it will be detected by CT. If it shows increased
glycolysis, but no anatomic abnormalities, it will be detected by PET.
[44] [85]

MAGNETIC RESONANCE IMAGING

Additional studies such as MRI to evaluate mediastinal structures, bone and brain scans to detect
metastatic disease, and staging mediastinoscopy are not performed routinely unless these tests
are indicated by specific symptoms or findings. MRI can accurately detect T 4 and metastatic
disease, especially disease involving the liver.
Comparing CT with MRI, the sensitivity and
specificity for metastatic detection are almost equal; however, CT scans cost less and are more
readily available than MRI scans. Bronchoscopy is imperative for esophageal carcinomas, which
are in proximity to the trachea or main stem bronchi (i.e., upper-third and middle-third tumors),
because endoscopic evidence of invasion of the airway precludes a safe esophagectomy. Videoassisted thoracoscopy and laparoscopy can provide direct access to thoracic or celiac lymph
nodes for biopsy or fine-needle aspiration. Major disadvantages of thoracoscopic or laparoscopic
staging are the requirements for general anesthesia, a double-lumen endotracheal tube, several
access ports, a 2 to 3-hour procedure, and a 2 to 3-day hospital stay with all associated costs and
risks.
[127]

THORACOSCOPY AND MINIMALLY INVASIVE STAGING

Video-assisted thoracoscopy to stage esophageal cancer has been shown to be highly accurate,
although invasive, in evaluating nodal status. Thoracoscopy allows visualization of the entire
thoracic cavity and esophagus from the thoracic inlet to the diaphragmatic hiatus, for biopsy of
lymph nodes as well as for visualization of the extent of local involvement. Thoracoscopy can
also visualize metastatic disease involving nearby or adjacent structures, such as the trachea,
azygos vein, aorta, pericardium, and diaphragm. A right-sided thoracoscopy is most commonly
performed so the esophagus can be viewed and manipulated without interference from the aorta.
A left-sided thoracoscopy is used when the patient has suspicious left-sided nodal findings,
especially aortopulmonary window nodes, from prior noninvasive radiologic techniques.
[77]

[74]

Celiac nodal involvement is common in patients with esophageal cancer, up to 46%, and also
predicts a poor prognosis. To overcome this limitation of thoracoscopy, laparoscopy has been
added as a complementary technique.
[43]

Laparoscopy is useful in evaluation and biopsy of the celiac axis, the surface of the peritoneal
cavity, the esophagogastric junction, and the liver. As a result, laparoscopy is routinely used to
complement thoracoscopy, to provide a method for accurate minimally invasive staging.
Laparoscopy is more sensitive than CT and ultrasound in the diagnosis of nodal and peritoneal
metastases. Laparoscopic ultrasound can visualize nodes as small as 3 mm in diameter with
resolution comparable to that of endoscopic ultrasound potentially to improve overall TNM
staging accuracy.
[75]

Treatment

Curative efforts include surgery, chemotherapy, radiation, or a combination of these techniques;

however, despite multitudes of clinical trials and retrospective reviews, no treatment modality
alone has proved superior. Current trials have focused on radiation and chemotherapy with or
without resection. Therapy for esophageal carcinoma is influenced by the knowledge that in most
of these patients, local tumor invasion or distant metastatic disease precludes cure. In fact, 85 to
95% of patients have lymph node involvement at the time of surgical resection. Fewer than 10%
of patients with lymph node involvement survive for 5 years. In the past, palliative techniques
were advocated because of the poor long-term survival rates of patients with esophageal
carcinoma. Palliation affords the patient the ability to swallow (at least saliva) and perhaps to
resume a normal life for 9 to 12 months. After the initial evaluation for staging, the physician can
assess whether palliative or curative approaches are indicated.
[146]

PALLIATIVE TREATMENT

Palliation is appropriate when patients are too debilitated to undergo surgery or have a tumor that
is unresectable because of extensive invasion of vital structures, recurrence of resected or
irradiated tumor, and/or metastases. Most of these patients have complete or partial obstruction
of the esophagus resulting from the tumor, and swallowing is painful or impossible. The goal of
palliation is to use the most effective and least invasive means possible to relieve dysphagia and
discomfort, to support nutrition, and to limit hospitalization. Palliation includes dilatation,
intubation, photodynamic therapy, radiotherapy with or without chemotherapy, surgery, and/or
laser therapy. None of these methods have proven superior.
Dilatation

Dilatation of malignant strictures to palliate dysphagia and to allow endoscopic ultrasound

evaluation is associated with a 2 to 3% risk of esophageal wall rupture or bleeding.
Unfortunately, relief is measured only in weeks. Patients with high-grade malignant strictures
more likely present with advanced disease. Similarly, 91% of patients with an obstructing tumor
precluding passage of an endoscope have stage III to IV disease.
[159]

Stenting

The purpose of a stent is to bridge the obstruction in the esophagus to allow luminal patency
primarily for control of saliva and secondarily for nutrition (Fig. 37-35) . Flexible, selfexpanding stents (Fig. 37-36) are constructed of two layers of superalloy monofilament wire
with a layer of silicon between them. The silicon sandwiched between the layers delays tumor
ingrowth through the holes in the wire mesh. After administration of local or general anesthesia,
the stricture is dilated to 42 to 45 French, the lesion is identified, and the expandable covered
stent is inserted under fluoroscopic or endoscopic control. Once the stent is inserted and
expanded, the ends flange out to anchor to the wall of the esophagus. Patients note chest
discomfort initially because of the stretching of the stricture. The insertion of self-expanding
metal stents does not preclude further treatment with chemotherapy or radiation.
The average survival after palliative intubation for esophageal carcinoma is less than 6 months.
Intraesophageal tube may permit oral alimentation for the several months of remaining life.
Photodynamic Therapy

For photodynamic therapy, a photosensitizer such as dihematoporphyrin ether, is given

intravenously and after 2 or 3 days is retained in the tumor in a much higher concentration than
in healthy tissue. Then, a low-power laser system that produces red light is delivered to the tumor
by a flexible endoscope. The photosensitizer absorbs the red light and produces oxygen radicals
to destroy the tumor. Two to 3 days after photodynamic therapy, esophagoscopy is repeated, and

the necrotic tumor tissue is removed, often monthly. Complications can include development of
fistulas and aspiration. Edema of the hands and face and sensitivity to sunlight after this therapy
are common complaints. Photodynamic therapy has high 5-year survival rates (62% in patients
with Stage I tumors), and some patients with Stage I tumors have experienced a complete
response. This form of therapy can be used in conjunction with chemotherapy and can be
repeated indefinitely.
[92]

[88]

Radiation Therapy

External-beam radiation relieves dysphagia in approximately 80% of the patients who undergo
therapy. In half of the patients, tumor regrowth occurs 6 months after radiation therapy has been
completed.
Intracavitary radiation does not affect the radiosensitive adjacent structures such as the lungs and
spinal cord that may be affected with external-beam therapy. Dysphagia-free survival can last up
to 12 months in 25 to 40% of patients. Complications are uncommon and include fibrotic
strictures, which can be effectively managed with dilatation.
[131]

Laser Therapy

Endoscopic laser therapy similarly improves dysphagia, but multiple treatments are required, and
long-term benefit is seldom achieved. The goal of this procedure is to produce necrosis of the
tumor with high-power (80 to 120 watts) and short-power durations of approximately 1 second
(range, less than 1 to 2 seconds) without administering general anesthesia. Treatments to reestablish luminal patency are required on average every 4 weeks (range, 3 to 10 weeks).
Morbidity and mortality risks with laser therapy are relatively low (less than 5%). Complications
include esophageal perforation, bacterial infection, abdominal distention, and either massive or
acute hemorrhage.
Surgical Palliation

In the past, a palliative surgical bypass with interposed stomach or colon was used when a tumor
was unresectable in a patient with severe dysphagia or when a tracheoesophageal fistula
occurred. Complications from this procedure included wound sepsis and anastomotic leaks. The
operative mortality was 11 to 40%. Postoperative death rates were much higher in patients with
cervical fistulas. Overall, of those patients returning home after surgery, most (75%) were able
to eat a full diet. Nevertheless, postoperative survival was only 1.5 to 14 months, with a mean
survival of 3 to 6 months.
[95]

[19]

[95]

An endothoracic endoesophageal pull-through operation consists of stripping the esophagus of

its mucosal layer and tumor and using the muscular tube of the esophagus as a sleeve through
which the stomach is pulled. Normal swallowing and normal diet are achieved in almost 80% of
the patients. Operative mortality rates are approximately 15%, and morbidity rates approach
25%. Complications include anastomotic and respiratory conditions.
[140]

CURATIVE TREATMENT

At best, only 50% of patients are eligible for a curative resection at presentation. The
lymphatic drainage of the esophagus is extensive, both within the esophageal wall and in the
surrounding mediastinal tissues. As a result, longitudinal extension of the esophageal carcinoma
may be extensive, and tumors may be multicentric. In 10% of patients, tumor recurs at the
resection margin in patients who have had a 6- to 8-cm margin of normal esophagus removed. If
an esophagectomy is indicated, three major technical approaches are available: (1) a
transthoracic esophagectomy, (2) transhiatal esophagectomy without a thoracotomy, and (3) an
]

[2]

en bloc radial esophagectomy. Although no consensus has been formed on the preferred
technique, transthoracic esophagectomy is preferred by most thoracic surgeons.
Regardless of technique, surgeons generally agree on the desirability of a so-called R0 resection
(i.e., a complete macroscopic and microscopic removal of tumor as the basic requirement in
surgery with curative intent for carcinoma of the esophagus and gastroesophageal junction).
Great controversy remains on the extent of the resection and the type of surgical access (i.e.,
transthoracic, left- or right-sided, or transhiatal resection). Some reports, especially from
Japanese groups, focus on the value of extended lymphadenectomy both in the mediastinum and
in the superior abdominal compartment (two-field lymphadenectomy). Many surgeons think that
adding bilateral cervical lymphadenectomy (three-field lymphadenectomy) is essential,
especially in patients with supracarinal tumors. As expected, these extensive resections and
reconstructions adversely affect surgical morbidity and mortality. Lymphadenectomy definitely
adds to improved pathologic staging. Some evidence indicates that extensive lymphadenectomy
improves prolonged disease-free survival times and cure rates through better control of local and
regional recurrence, which may result in better control of distant metastases. As a result, even in
patients with advanced Stage III disease, 5-year survival rates of around 20% can be obtained
after an R0 resection.
Transthoracic Esophagectomy

Transthoracic esophagectomy is still preferred by most thoracic surgeons because it allows

complete lymph node dissection under direct vision, complete resection of tumor mass and
adjacent tissue, and complete staging of the tumor. The esophagus lies on the right side of the
mediastinum, except in the most distal third, where it bends to the left. Moreover, the aortic arch
overlies the left side of the upper esophagus and obscures visibility during resection of a tumor
in the middle to upper third. The traditional surgical approach to distal esophageal carcinoma has
been a left-sided thoracoabdominal incision (Fig. 37-37) (Figure Not Available) . The distal
esophagus, proximal stomach, and adjacent lymph node-bearing tissues are resected, and an
intrathoracic esophagogastric anastomosis is performed. For higher thoracic esophageal tumors,
a thoracoabdominal incision or separate thoracic and abdominal incisions are used, and a high
intrathoracic esophagogastric anastomosis is performed. Unfortunately, a combined thoracic and
abdominal operation in a debilitated patient may lead to respiratory insufficiency, resulting from
postoperative incisional pain and an inability to breath deeply, that requires prolonged
mechanical ventilatory assistance and often causes death.
Disruption of an intrathoracic
esophageal anastomosis results in mediastinitis and sepsis, fatal in 50% of the patients. An
additional disadvantage of the intrathoracic esophageal anastomosis is inadequate long-term
relief of dysphagia either because of anastomotic suture-line tumor recurrence or because of the
development of reflux esophagitis above the anastomosis. Finally, intrathoracic esophagogastric
anastomoses are almost invariably associated with the development of reflux esophagitis, which
follows disruption of the LES mechanism. The operative mortality varies significantly, ranging
from as high as 14% to as low as 2.2%.
[46]

[18]

[32]

The posterior lateral thoracotomy incision is made (on the right, the fifth intercostal space is
entered, and on the left, the sixth to seventh). Then, an upper midline laparotomy and, if the
tumor is in the upper third, a left neck incision are made. The lung and pleural space are
examined for any evidence of metastatic disease.
The inferior pulmonary ligament is divided to the inferior pulmonary vein. The tumor area is
then examined for any evidence of direct invasion of any vital or unresectable mediastinal
structures. The esophagus, periesophageal lymphatics, and adjacent pleura are resected,
preferably en bloc. The paratracheal lymph nodes are also removed. Great care is taken to avoid
any damage to the recurrent laryngeal nerve, to avoid hoarseness. The azygos vein and the

thoracic duct are resected along with the primary specimen. The opposing pleura is not resected
unless it appears to be invaded with tumor. If dissection of the esophagus at the thoracic inlet has
been adequate, the esophagus should be easily mobilized from the anterior longitudinal ligament
of the spine. The esophagus is then transected 5 to 8 cm from the UES, yet a sufficient distance
away from the primary tumor, at least 5 cm but usually 10 cm, to avoid skip metastases or
longitudinal lymphatic spread. The esophagogastric anastomosis may be performed in a single
layer or a double layer, or with an end-to-end stapling device or end-to-side stapling system.
[3] [116]

The most direct route for the conduit of reconstruction (stomach, colon, roux-en-Y loop of
jejunum) is the posterior mediastinum in the prevertebral space created by the resected
esophagus. Some investigators have advocated placing the neoesophagus in a substernal position
to reduce the likelihood of a local recurrence that causes obstruction. For distal-third tumors
that are located at the esophageal hiatus and the diaphragm, a left thoracotomy alone allows a
sufficient amount of diaphragm to be resected with the specimen to achieve a negative margin. A
cephalad transection site is then chosen approximately 10 cm above the most superior portion of
the esophageal tumor. The gastric margin is approximately 5 cm from the lowest portion. The
remaining stomach is then pulled up into the retromediastinum, and an anastomosis is performed
(end-to-end or end-to-side anastomosis) using either a single-layer or a two-layered hand-sewn
anastomosis or stapling devices.
[71]

A total thoracic esophagectomy is similar, but plans include removal of the entire esophagus to
maximize the resection margin. This procedure begins with a laparotomy, as do all
esophagectomies, to mobilize the conduit of choice. A right-sided thoracotomy is then made, and
the esophagus is resected from a 5-cm gastric margin at the cardia to within 2 to 3 cm of the
UES. The conduit, whether it be the stomach or the colon, is placed either retrosternally or in the
original esophageal bed, and a cervical anastomosis is performed.
En Bloc Esophagectomy

Because many patients present with metastases to regional lymph nodes as well as to the
surrounding tissue and organs, a more radical resection, the en bloc esophagectomy, has been
advocated by a few thoracic surgeons. An envelope of normal tissue is removed along with the
spleen, celiac nodes, posterior pericardium, azygos vein, thoracic duct, and adjacent diaphragm
(Fig. 37-39 (Figure Not Available) and Table 37-12) (Table Not Available) . With this aggressive
surgery, operative mortality ranges from 5.1 to 11%, not significantly different from other
approaches.
The two major complications are similar to transhiatal and transthoracic
esophagectomy: anastomotic leak and respiratory complications. With the en bloc technique, 5year survival rate is 40 to 55% for patients with Stage I adenocarcinoma confined to the
esophageal wall. In adenocarcinoma, increased incidence of regional lymph node metastases
has been reported with increasing depth of invasion of the primary tumor. Lymph nodes are
involved in 80% of patients with muscular invasion. Some surgeons advocate a three-field
dissection (bilateral cervical, mediastinal, and abdominal) followed by esophagectomy for
patients with locally advanced carcinoma of the thoracic esophagus in the presence of lymph
node metastasis; 5-year survival is 42% and up to 54% in patients with fewer than four positive
nodes.
[7]

[7]

[142]

Transhiatal Esophagectomy

Because of the risks associated with the more radical transthoracic or en bloc esophagectomies
and the overall low survival rate of patients with esophageal carcinomas, transhiatal
esophagectomy without thoracotomy was proposed. In this operation, regardless of the level of
the tumor, the entire thoracic esophagus is resected and replaced, whenever possible, with the

stomach anastomosed to the remaining cervical esophagus above the level of the clavicles (Fig.
37-40) .
Advocates of transhiatal esophagectomy report a low operative mortality of 2 to 8% and a low
anastomotic leak rate of 5 to 7.9%.
However, other studies of this operation reported
a higher anastomotic leak rate of 26%, with similar patient morbidity and mortality compared
with transthoracic and en bloc esophagectomy.
In performing a transhiatal esophagectomy,
the surgeon removes accessible cervical, intrathoracic, and intra-abdominal lymph nodes for
staging, but a complete en bloc resection of adjacent lymph node-bearing tissue is not
accomplished. The advantages of this approach are as follows: (1) a thoracotomy is avoided, thus
minimizing the physiologic insult of the operation; (2) an intrathoracic esophageal anatomosis is
avoided, and if a cervical leak does occur, it is more easily managed and rarely causes
mediastinitis or fatal complications; (3) no intra-abdominal or intrathoracic gastrointestinal
suture lines are present; and (4) clinically significant gastroesophageal reflux seldom occurs after
a cervical esophagogastric anastomosis. Contraindications to the transhiatal approach include
evidence of tumor invasion of the pericardium, aorta, and/or tracheobronchial tree.
[50] [58] [97] [121] [162]

[58] [154]

[121]

Some early and late complications associated with the transhiatial approach are wound infection,
anastomotic leak, respiratory complications, pneumothorax, recurrent laryngeal nerve injury,
esophageal stricture, and delayed gastric emptying.
The transhiatal esophagectomy is performed through an upper-midline abdominal and cervical
incision without thoracotomy; therefore, the thoracic esophagus is resected through the
diaphragmatic hiatus and the neck. The stomach is mobilized by dividing the left gastric and left
gastroepiploic vessels, and the right gastric and the right gastroepiploic arcades are preserved
(Fig. 37-41) . Pyloromyotomy and feeding jejunostomy are performed routinely. The entire
thoracic esophagus from the level of the clavicles to the cardia is resected, while one carefully
monitors intra-arterial blood pressure to avoid prolonged hypotension from cardiac displacement
during the transhiatal esophageal dissection (Fig. 37-42) . The surgical stapler is used to fashion
a gastric tube from the greater curvature (Fig. 37-43) while still preserving its entire length. The
stomach is mobilized through the posterior mediastinum in the original esophageal bed (Fig. 3744) and is anastomosed (hand sewn or stapled) to the cervical esophagus (Fig. 37-45) . The
normal stomach readily reaches to the neck in every patient. For distal-third esophageal tumors
localized to the cardia, the high lesser curvature of the stomach is resected 4 to 6 cm beyond the
gross tumor, to preserve that point on the high greater curvature that reaches cephalad to the neck
(Fig. 37-46) for the cervical esophagogastric anastomosis. Even relatively large intrathoracic
esophageal carcinomas are resectable through the enlarged hiatus. For tumors of the upperthoracic esophagus, the addition of a partial upper sternal split facilitates dissection of the
esophagus from the trachea under direct vision (Fig. 37-47) .
[113]

Thoracoscopic Esophagectomy

Several authors have reported the use of video-assisted thoracoscopy or laparoscopy in

performing esophagectomy.Thoracoscopic esophagectomy has three stages. The first is the
thoracoscopic dissection of the thoracic esophagus. The second is the laparoscopic mobilization
of the intended gastric conduit, and the third is the cervical anastomosis.
Major causes of complications include respiratory disorders, anastomotic leak, and laryngeal
nerve injury. Some investigators have concluded that, at this early stage, this procedure has no
advantage over open surgical procedures.
[80]

Reconstruction After Esophagectomy

After a portion of the esophagus is removed, or after complete esophagectomy, a conduit must be
established for alimentary continuity. The stomach, colon, and jejunum have all been
successfully used as esophageal substitutes (Figs. 37-48 and 37-49) , but the stomach appears to
be the conduit of choice because of ease in mobilization and its ample vascular supply (Fig. 3750) (Figure Not Available) . A higher incidence of mortality is noted with the use of the colon
because of the necessity for three anastomoses (coloesophagostomy, colojejunostomy, and
colocolostomy). The colon is used if the patient has undergone a partial or total gastrectomy
previously or if tumor involves the stomach . Jejunal loops can also be used, but their limited
vascular supply restricts mobility (Fig. 37-52) .
Anastomosis can be performed in the chest just below the arch of the aorta (intrathoracic
anastomosis), or a cervical anastomosis can be made in the neck, depending on the choice of
reconstruction. Mechanical staplers continue to improve, and leak rates are decreasing. Leakage
is more likely to occur in patients who are malnourished, in those who have had preoperative
radiation therapy, and in those who have tension at the anastomosis. A leak most frequently
occurs within 10 days of the surgical procedures, often at the time of the first contrast swallow
examination. Patients with a leak may also present with signs of sepsis or increased drainage
output from previously placed chest tubes and drains. For a cervical anastomotic leak, a
conservative approach is advised. The drainage can be controlled by opening the cervical
incision to create a cervical fistula.
With adequate drainage, the leak usually spontaneously
closes within 1 to 2 weeks and mortality is rare. Nutritional support is maintained by an enteral
feeding tube. Approximately half of the patients who have an anastomotic leak develop a
stricture relieved by serial esophageal dilatation. For small leaks that are well drained, the patient
may be managed with antibiotics, nutritional support, and close observation. Leaks from an
anastomosis in the mediastinum are significantly more serious, with mortality rates of 20 to 40%.
For an intrathoracic anastomotic leak, in most cases, reoperation should be performed. The
anastomosis should be inspected. If repair seems feasible, it may be attempted. Usually, however,
the safest option is to take down the anastomosis and mobilize the remaining esophagus out of
the chest through a cervical incision for construction of an anterior thoracic end-esophagostomy
(Fig. 37-53) (Figure Not Available) . Devitalized stomach is resected, and the remaining stomach
is returned to the abdominal cavity. A decompressing gastrostomy is performed. The pleural
cavity and mediastinum should be debrided, thoroughly irrigated, and adequately drained. Future
reconstruction with a colon interposition remains an option.
[15]

[116]

Laryngopharyngectomy for cervicothoracic tumors and concomitant transhiatal esophagectomy

without thoracotomy provide the maximum distal esophageal margin beyond the tumor and
permit restoration of continuity of the alimentary tract. However, a colon interposition is the best
means of restoring alimentary continuity in this situation, as regurgitation after a pharyngogastric
anastomosis gives a less satisfactory functional result.
Preoperative Preparation for Esophagectomy

If the patient is dehydrated or if the esophageal obstruction is tight, endoscopic dilatation of the
malignant stricture and insertion of a nasogastric feeding tube or an intraluminal stent for enteral
nutrition are performed to achieve an intake of approximately 2000 calories per day. Intravenous
hyperalimentation is seldom indicated, because of the associated septic and metabolic
complications. Oral hygiene is often neglected, and abscessed or severely carious teeth should be
removed or repaired preoperatively to minimize the severity of an infection that may result from
anastomotic disruption and swallowed oral bacteria. If the patient has a history of prior gastric
operations that may preclude the use of the entire stomach as an esophageal substitute, a barium
enema examination should be done to assess the suitability of the colon for esophageal

replacement, and the colon should be prepared in the event that a colonic interposition is
required.
Radiation Therapy

The goals of preoperative radiation therapy are to reduce the tumor size, to control the amount of
local spread of the tumor before surgery, and to reduce the risk of tumor spread at the time of
surgical manipulation. Preoperative radiation therapy does not significantly improve 5-year
survival after surgery. The goal of postoperative radiation therapy is to destroy residual
malignant cells after surgical resection, especially if positive tumor margins are discovered after
resection. Despite improvement in local recurrence, no improvement in survival has been
realized.
[2]

Chemotherapy

Chemotherapy as a single modality in the treatment of esophageal cancer is the least effective
strategy. Although radiographic improvement can be seen in up to one-half of patients, two or
three cycles (6 to 12 weeks) of chemotherapy are required, relief of dysphasia is slow and/or
incomplete, and survival is anecdotal. Unfortunately, no reliable method exists to identify
"responders" before therapy is begun. Chemotherapy is used preoperatively alone or in
combination with radiation therapy to treat micrometastases and to reduce the size of the tumor
to improve resectability rate. Moreover, if surgery is not appropriate, chemotherapy is used with
radiation therapy for palliation and possibly to improve survival. Chemotherapy is typically
given in a combination of two or more drugs.
Barrett's Esophagus
Injured squamous cells in the distal esophagus can be replaced either by more squamous cells or,
through the process of metaplasia, by columnar cells (Barrett's esophagus). Chronic
gastroesophageal reflux both injures the squamous epithelium and provides the abnormal
esophageal environment that stimulates repair through columnar cell metaplasia. Up to three
different types of columnar epithelia can be found in Barrett's esophagus: (1) specialized
intestinal metaplasia; (2) gastric fundic- type epithelium; and (3) junctional-type epithelium.
Specialized intestinal metaplasia is the most common, and dysplasia and carcinoma in Barrett's
esophagus are almost invariably associated with specialized intestinal metaplasia.
Diagnosis

Barrett's esophagus is more common in men than in women, with a 3:1 male predominance (the
average age at diagnosis is 55 years). The prevalence of Barrett's esophagus increases with age
up to 70 years. Barrett's esophagus often remains stable, and no conclusive evidence indicates
that either ongoing severe reflux or effective treatment of this condition alters the progession of
Barrett's esophagus despite the association with cancer. The extent of intestinal metaplasia is
related to the status of the LES and the degree of esophageal acid exposure.
Barrett's
esophagus can be found in 10 to 15% of patients who have endoscopic examinations for
symptoms of GERD. Most patients with Barrett's esophagus do not seek medical attention for
esophageal symptoms and may have no symptoms of GERD. The GERD associated with
Barrett's esophagus, however, often is severe, with esophageal ulceration, stricture, and
hemorrhage. Barrett's esophagus has been identified in approximately 1 in 10 persons with
erosive esophagitis and 1 in 3 persons with a peptic esophageal stricture. In one study, small
areas of specialized columnar epithelium with intestinal metaplasia were identified histologically
in the region of the gastroesophageal junction in 18% of patients undergoing endoscopy. This
[108]

finding indicates that "short segment Barrett's esophagus" may be common in the general
population
Adenocarcinomas now account for approximately one third of all esophageal malignancies. Most
of these tumors arise from Barrett's epithelium.
True dysplasia in Barrett's esophagus represents a neoplastic alteration of the columnar
epithelium and is widely regarded as the precursor of invasive malignancy. Unfortunately,
dysplasia is not an ideal biomarker of malignant potential in Barrett's epithelium for several
reasons. The histologic interpretation of dysplasia is largely subjective, and the natural history of
dysplasia is not clear. Dysplastic Barrett's mucosa often is indistinguishable from nondysplastic
mucosa, and small foci of dysplasia can be easily missed. Despite limitations, dysplasia remains
the best biomarker for evaluating malignancy in Barrett's esophagus. Some studies have
indicated that approximately one third of patients with high-grade dysplasia in Barrett's
esophagus either already have or will develop invasive cancer within several years. The
prevalence of adenocarcinoma at the time of diagnosis of Barrett's esophagus is approximately
8%.
Treatment

Patients undergoing surgery for carcinoma in situ or confirmed high-grade dysplasia should have
an esophagectomy that includes the entire columnar-lined esophageal segment. The reasons for
this recommendation are twofold. First, Barrett's esophagus is a premalignant condition, and
high-grade dysplasia or carcinoma may develop subsequently in any columnar-lined tissue that
remains after surgery. Second, some studies have reported that as many as 50% of patients who
undergo esophagectomy for high-grade dysplasia have an unrecognized adenocarcinoma in the
surgical specimen. Multicentric cancers are common, occurring in 13 to 37% of resection
specimens for Barrett's esophageal adenocarcinoma.