gus include expansion of the Ki-67 proliferation compartment correlating with the degree of intraepithelial neoplasia {738}. Molecules involved in membrane trafficking such as rab11 have been reported to be specific for the loss of polarity seen in low-grade intraepithelial neoplasia {1566}. In invasive carcinoma, reduced expression of cadherin/catenin complex and increased expression of various proteases are detectable. Non-neoplastic Barrett oesophagus expresses the MUC2 but not the MUC1 mucin gene product, whereas neither is expressed in intraepithelial neoplasia in Barrett oesophagus {298}. Invasive lesions exhibit variable expression of MUC1 and MUC2.

Prognostic factors

The major prognostic factors in adenocarcinoma of the oesophagus are the depth of mural invasion and the presence or absence of lymph node or distant metastasis {734, 1049, 1458, 1945}. Gross features and histological differentiation do not influence prognosis. The overall 5-year survival rate after surgery is less than 20% in most series including a majority of advanced carcinomas. The survival rates are better in superficial (pT1) adenocarcinoma, ranging from 65% to 80% in different series {735, 1219}.

Since the stage at the time of diagnosis is the most important factor affecting outcome, endoscopic surveillance of Barrett patients with early detection of their ade-nocarcinomas, results in better prognosis in most cases {1995}.

Endocrine tumours of the oesophagus

C. Capella E. Solcia L.H. Sobin R. Arnold


Endocrine tumours of the oesophagus are rare and include carcinoid (well differentiated endocrine neoplasm), small cell carcinoma (poorly differentiated endocrine carcinoma), and mixed endocrine-exocrine carcinoma.

ICD-O codes

Carcinoid 8240/3

Small cell carcinoma 8041/3 Mixed endocrine-exocrine carcinoma 8244/3


In an analysis of 8305 carcinoid tumours of different sites, only 3 (0.04%) carci-noids of the oesophagus were reported {1251}. They represented 0.05% of all gastrointestinal carcinoids reported in this analysis and 0.02% of all oesophageal cancers. All cases were in males and presented at a mean age of 56 years {1251}. Small cell carcinoma occurs mainly in the sixth to seventh decade and is twice as common in males as females {190, 421, 765, 1026}. The reported frequencies among all oesophageal cancers were between 0.05% to 7.6 % {190, 421, 765, 1026}.

The few mixed endocrine-exocrine carcinomas were in males at the sixth decade {256, 301}.

Aetiological factors

Patients with small cell carcinomas often have a history of heavy smoking and one reported case was associated with long standing achalasia {93, 1539}. A case of combined adenocarcinoma and carci-noid occurred in a patient with a Barrett oesophagus {256}. Small cell carcinoma has also been associated with Barrett oesophagus {1678, 1813}.


Carcinoid tumours are typically located in the lower third of the oesophagus {1329, 1567, 1754}. Almost all small cell carcinomas occur in the distal half of the oesophagus {190, 421}.

Clinical features

Dysphagia, severe weight loss and sometimes chest pain are the main symptoms of endocrine tumours of the oesophagus. Patients with small cell carcinomas often present at an advanced stage {765, 1026}. Inappropriate antidiuretic hormone syndrome and hypercalcemia have been reported {421}. In addition, a case of watery diarrhoea, hypokalaemia-achlor-hydria (WDHA) syndrome, due to ectopic production of VIP by a mixed-cell (squamous-small cell) carcinoma of the oesophagus has been described {2070}.

Fig. 1.30 Small cell carcinoma of the oesophagus.


All reported oesophageal carcinoids were of large size (from 4 to 7 cm in diameter) and infiltrated deeply the oeso-phageal wall {1329, 1567, 1754}. Small cell carcinomas usually appear as fun-gating or ulcerated masses of large size, measuring from 4 to 14 cm in greatest diameter.


Carcinoid (well differentiated endocrine neoplasm)

All carcinoids so far reported in the literature have been described as deeply infil-trative tumours, with high mitotic rate and metastases {1329, 1567, 1754}. Microscopically, they are composed of solid nests of tumour cells that show positive stain for Grimelius and neuron-specific enolase {1567}, and characteristic membrane-bound neurosecretory granules at ultrastructural examination {1754}.

Small cell carcinoma (poorly differentiated endocrine carcinoma)

Small cell carcinoma of the oesophagus is indistinguishable from its counterpart


Primary lymphoma of the oesophagus is defined as an extranodal lymphoma arising in the oesophagus with the bulk of the disease localized to this site {796}. Contiguous lymph node involvement and distant spread may be seen but the primary clinical presentation is in the oesophagus with therapy directed at this site.

Clinical features

The oesophagus is the least common site of involvement with lymphoma in the digestive tract, accounting for less than 1% of lymphoma patients {1399}. Oeso-phageal involvement is usually second in the lung according to histological and immunohistochemical features as well as clinical behaviour. The cells may be small with dark nuclei of round or oval shape and scanty cytoplasm, or be larger with more cytoplasm (intermediate cells) forming solid sheets and nests. There may be foci of squamous carcinoma, adenocarcinoma, and/or mucoepi-dermoid carcinoma, a finding that raises the possibility of an origin of tumour cells from pluripotent cells present in the squamous epithelium or ducts of the submucosal glands {190, 1887}. Argyro-phylic granules can be demonstrated by Grimelius stain, and small dense-core granules are always detected by electron microscopy {781}. Immunohistochemical reactions for neuron-specific enolase, synaptophysin, chromogranin and leu7 usually are positive and represent useful diagnostic markers {723}. Some cases have been associated with calcitonin and ACTH production {1272}.

Mixed endocrine-exocrine carcinoma

In the few reported cases {256, 301}, the ary either from the mediastinum, from nodal disease or from a primary gastric location. Patients are frequently male and usually over 50 years old. Tumours involving the distal portion of the oesophagus may cause dysphagia {644}.


Primary oesophageal lymphomas may be of the large B-cell type or may be low-grade B-cell MALT lymphomas {1794}. MALT lymphomas show morphological and cytological features common to MALT lymphomas found elsewhere in the digestive tract. Lymphoid follicles are surrounded by a diffuse infiltrate of cen-trocyte-like (CCL) cells showing a vari tumours combined a gastrointestinal-type adenocarcinoma with the trabecu-lar-acinar component of a carcinoid. In one case the carcinoid component was positive for Grimelius stain, Fontana argentaffin reaction and formaldehyde induced fluorescence for amines {301}.

Prognostic factors

Two of three oesophageal carcinoids from the analysis of 8305 cases of carci-noid tumours {1251} were associated with distant metastases and one {1567} of the three reported cases {1329, 1567, 1754} died 29 months after surgery. The prognosis of small cell carcinoma of the oesophagus is poor, even when the primary growth is limited {190, 421}. The survival period is usually less than 6 months {816 and thus similar to that of patients with small cell carcinoma of the colon {765, 1026}. Multidrug chemotherapy may offer temporary remission {765, 816, 1026, 1678}.

A. Wotherspoon A. Chott R.D. Gascoyne H.K. Muller-Hermelink able degree of plasma cell differentiation. Infiltration of these cells into the overlying epithelium is usually seen. Characteristically the CCL cells express pan-B-cell markers CD20 and CD79a and they are negative for CD5 and CD10. They express bcl-2 protein and may be positive with antibodies to CD43. Due to the rarity of these lesions, molecular genetics data are not available. In common with other sites in the digestive tract, secondary involvement of the oesophagus may occur in dissemination of any type of lymphoma. Primary oesophageal T-cell lymphoma has been described but is exceedingly rare {547}.

Lymphoma of the oesophagus

Mesenchymal tumours of the oesophagus


A variety of rare benign and malignant mesenchymal tumours that arise in the oesophagus. Among these, tumours of smooth muscle or 'stromal' type are most common.

ICD-O codes

Leiomyoma 8890/0

Leiomyosarcoma 8890/3 Gastrointestinal stromal tumour (GIST) 8936/3

Granular cell tumour 9580/0

Rhabdomyosarcoma 8900/3

Kaposi sarcoma 9190/3


The morphological definitions of these lesions follow the WHO histological classification of soft tissue tumours {2086}. Stromal tumours are described in detail in the chapter on gastric mesenchymal tumours.

as females and has a median age distribution between 30 and 35 years {1712, 1228}. Sarcomas of the oesophagus accounted for 0.2% of malignant oesophageal tumours in SEER data from the United States from 1973 to 1987. Males were more frequently affected than females by nearly 2:1 {1928}. Adults between the 6th and 8th decades are primarily affected. Oesophageal stromal tumours show demographics similar to those of sarcomas {1228}.


Leiomyomas and stromal tumours are most frequent in the lower oesophagus and begin as intramural lesions. The larger tumours can extend to mediastinum and form a predominantly mediastinal mass. Leiomyomatosis forms worm-like intramural structures that may extend into the upper portion of the stomach.


Leiomyoma is the most common mesenchymal tumour of the oesophagus. It occurs in males at twice the frequency

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