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Review
. 2021 Feb;113(1):5-11.
doi: 10.32074/1591-951X-229.

Neuroendocrine neoplasms of the esophagus and stomach

Luca Mastracci  1   2 , Guido Rindi  3   4 , Federica Grillo  1   2 , Enrico Solcia  5 , Michela Campora  1 , Matteo Fassan  6 , Paola Parente  7 , Alessandro Vanoli  5 , Stefano La Rosa  8
Affiliations
Review

Neuroendocrine neoplasms of the esophagus and stomach

Luca Mastracci et al. Pathologica. 2021 Feb.

Abstract

Esophageal neuroendocrine neoplasms (E-NENs) are much rarer than other gastro-entero-pancreatic neuroendocrine neoplasms, the majority showing aggressive behavior with early dissemination and poor prognosis. Among E-NENs, exceptionally rare well differentiated neuroendocrine tumors (E-NET) and more frequent esophageal poorly differentiated neuroendocrine carcinomas (E-NEC) and mixed neuroendocrine-non neuroendocrine neoplasms (MiNEN) can be recognized. E-NECs usually exhibit a small cell morphology or mixed small and large cells. Esophageal MiNEN are composed of NEC component admixed with adenocarcinoma or squamous cell carcinoma. Gastric (G) NENs encompass a wide spectrum of entities ranging from indolent G-NETs to highly aggressive G-NECs and MiNENs. Among G-NETs, ECL-cell NETs are the most common and, although composed of histamine-producing cells, are a heterogeneous group of neoplastic proliferations showing different clinical and prognostic features depending on the patient's clinico-pathological background including the morphology of the peri-tumoral mucosa, gastrin serum levels, presence or absence of antral G-cell hyperplasia, and presence or absence of MEN1 syndrome. In general, NET associated with hypergastrinemia show a better outcome than NET not associated with hypergastrinemia. G-NECs and MiNENs are aggressive neoplasms more frequently observed in males and associated with a dismal prognosis.

Keywords: MiNEN; esophagus; neuroendocrine carcinoma; neuroendocrine neoplasms; neuroendocrine tumor; stomach.

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Conflict of interest statement

Conflict of interest

The Authors declare no conflict of interest.

Figures

Figure 1.
Figure 1.
(A) Esophageal small cell neuroendocrine carcinoma undermining normal squamous esophageal mucosa; H&E, magnification 4x. (B) Small cell neuroendocrine carcinoma infiltrating the muscular wall of the esophagus; H&E, magnification 10x. (C) Diffuse positivity of neoplastic cells for synaptophysin, magnification 20x. (D) Faint, dot-like, positivity for Chromogranin A; magnification 20x. (E) High proliferative index, 80% (Ki-67 stain), magnification 20x.
Figure 2.
Figure 2.
Type 1 ECL-cell NET is composed of well-differentiated cells arranged in small microlobular and/or trabecular structures (A, bottom). The peritumoral oxyntic mucosa (A, top) is atrophic with diffuse intestinal metaplasia and shows ECL-cell linear and micronodular hyperplasia, easily detected with chromogranin A immunostaining (B). Type 3 NETs is composed of well differentiated neuroendocrine cells as well, deeply invading the gastric wall (C, right). Peritumoral oxyntic mucosa is normal (C, left) without ECL-cell proliferations (D).
See this image and copyright information in PMC

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