Neuroendocrine neoplasms of the esophagus and stomach

Luca Mastracci, Guido Rindi, Federica Grillo, Enrico Solcia, Michela Campora, Matteo Fassan, Paola Parente, Alessandro Vanoli, Stefano La Rosa

Research output: Contribution to journalReview articlepeer-review

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.

Original languageEnglish
Pages (from-to)5-11
Number of pages7
JournalPathologica
Volume113
Issue number1
DOIs
Publication statusPublished - Feb 1 2021

Keywords

  • esophagus
  • MiNEN
  • neuroendocrine carcinoma
  • neuroendocrine neoplasms
  • neuroendocrine tumor
  • stomach

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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