Clinicopathological Features of Gastroesophageal Neuroendocrine Neoplasms
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STOMACH AND DUODENUM (J PISEGNA AND J BENHAMMOU, SECTION EDITORS)
Clinicopathological Features of Gastroesophageal Neuroendocrine Neoplasms Mengyuan Liu 1 & Elizabeta C. Popa 2 & Brendan M. Finnerty 1 & Thomas J. Fahey III 1 & Rasa Zarnegar 1,3
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review Gastroesophageal neuroendocrine neoplasms (NENs) are a rare entity. Recent 2019 WHO classifications reflect our understanding of tumor biology, namely, that distinct molecular characteristics underline tumor behavior and prognosis. Here, we reviewed the evidence for linking molecular findings with the clinicopathological features and treatment of gastroesophageal NENs. Recent Findings Degree of differentiation and Ki-67 proliferation index are required for accurate classification of neuroendocrine tumors and carcinomas but not sufficient to distinguish between the two entities. Resection remains the mainstay treatment for early-stage gastroesophageal neuroendocrine tumors. Additional perioperative therapy may benefit mitotically active tumors. There is a role for somatostatin analogues, especially in the setting of metastatic and symptomatic disease. New radiolabeled somatostatin analogues, immunotherapy, and embolization offer multimodality treatments for distant metastases. Summary We need to understand the specific underlying biology of the various subtypes of gastroesophageal NENs to provide tailored treatment. Keywords Gastric . Esophageal . Neuroendocrine tumors . Mixed neuroendocrine-neuroendocrine neoplasms . Somatostatin analogues
Introduction Over 70% of all neuroendocrine neoplasms (NENs) are found in the digestive system. The stomach and esophagus are among the least common locations for NENs with incidence of 10% and < 1% at each site, respectively [1, 2].
Mengyuan Liu and Elizabeta C. Popa contributed equally to this work. This article is part of Topical Collection on Stomach and Duodenum * Rasa Zarnegar [email protected] 1
Department of Surgery, Division of Endocrine & Minimally Invasive Surgery, Weill Cornell Medical College, New York-Presbyterian Hospital, New York, NY, USA
2
Department of Medicine, Division of Hematology/Oncology, Weill Cornell Medical College, New York-Presbyterian Hospital, New York, NY, USA
3
Department of Surgery, Division of Endocrine and Minimally Invasive Surgery, Weill Cornell Medicine, 525 East 68th Street, K-836, New York, NY 10065, USA
Updated retrospective, population-based Surveillance, Epidemiology, and End Results (SEER) data reveal an increasing incidence of NENs at all gastrointestinal sites with an annual percentage rate change of over 5% between 1986 and 2015 [3]. Gastric NENs have had an overall 15-fold increase from 1973 to 2012, while esophageal NENs are too rare to be adequately analyzed. This increased incidence may be due to use of endoscopy and discovery of early-stage tumors, with improved imaging. Survival of patients with advanced staged tumors has also improved [4]. The 5th edition of the WHO classif
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