Gastric neuroendocrine tumors: A review of pathology and updated roadmap to surgical management

Abstract

Neuroendocrine tumors are a rare cancer, with those arising in gastric tissue even less commonly. With increasing recognition through endoscopy, these tumors are diagnosed in more patients each year. As a rare and growing entity, our understanding of these tumors, the way we characterize them, and treatment are changing rapidly. Thus, we sought to provide an updated review of pathology and management, highlighting the latest guidelines and evidence for surgical treatment. Much of the general treatment paradigm is from consensus guidelines put forth by the European Neuroendocrine Tumor Society and the North American Neuroendocrine Tumor Society; however, future research is needed to help guide further surgical decision-making around intermediate grade and intermediate size type III tumors, as well as systemic therapies in the perioperative and nonoperative settings for high-grade tumors.

Keywords: Gastric; Carcinoid; Neuroendocrine tumor; Stomach; Endoscopic; Surgical management

Core Tip: Gastric neuroendocrine tumors are a rare pathology, with rising incidence as endoscopic diagnoses increase. Management, including surgical decision-making, is guided by subtype as well as tumor size and grade, which have been shown to be prognostic. There still exist areas that lack consensus, namely for extent of surgery for intermediate-grade and medium-sized type III tumors, as well as in clarifying the role of systemic therapies for high-grade tumors in the perioperative settings and for nonoperative metastatic disease specifically for gastric-originating tumors. Existing practice guidelines provide surgeons the framework for treatment, however future work is needed to fill gaps in decision-making consensus.