Overview

NCI Definition: A well-differentiated neuroendocrine tumor arising from the digestive system. It is characterized by the presence of cells with features similar to those of the normal endocrine cells of the digestive system. The neoplastic cells express immunohistochemical evidence of neuroendocrine differentiation and hormones. There is mild to moderate nuclear atypia and less than 20 mitoses per 10 HPF. It includes well-differentiated endocrine tumors or carcinoid tumors and well-differentiated endocrine carcinomas. [1]

Gastrointestinal neuroendocrine tumorss most frequently harbor alterations in TP53, MEN1, CDKN1B, KMT2A, and BCOR [2].

Most Commonly Altered Genes in Gastrointestinal Neuroendocrine Tumors

TP53 Mutation, TP53 c.217-c.1178 Missense, TP53 Missense, BCOR Mutation, and APC Mutation are the most common alterations in gastrointestinal neuroendocrine tumors [2].

Top Alterations in Gastrointestinal Neuroendocrine Tumors

Disease Details

Synonyms
Digestive System Neuroendocrine Tumor, Gastrointestinal System Neuroendocrine Tumor, Digestive System Well Differentiated Neuroendocrine Tumor, GEP-NET, Gastroenteropancreatic NET, Gastrointestinal NET, Digestive System NET, Gastrointestinal Neuroendocrine Tumor, Gastroenteropancreatic Neuroendocrine Tumor
Parent(s)
Neuroendocrine Tumor
Children
Somatostatin-Producing Neuroendocrine Tumor, Pancreatic Neuroendocrine Tumor, Colorectal Neuroendocrine Tumor, Gastric Neuroendocrine Tumor, Gastrin-Producing Neuroendocrine Tumor, Small Intestinal Neuroendocrine Tumor, VIP-Producing Neuroendocrine Tumor, L-Cell Glucagon-Like Peptide-Producing Neuroendocrine Tumor, Appendix Neuroendocrine Tumor, and Esophageal Neuroendocrine Tumor
OncoTree Name
Gastrointestinal Neuroendocrine Tumors
OncoTree Code
GINET

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.