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, KRAS, CDKN2A, and ATM [2].

Most Commonly Altered Genes in Gastrointestinal Neuroendocrine Tumors

TP53 Mutation, TP53 c.217-c.1178 Missense, TP53 Missense, MEN1 Mutation, and ATM Mutation are the most common alterations in gastrointestinal neuroendocrine tumors [2].

Top Alterations in Gastrointestinal Neuroendocrine Tumors

Disease Details

Synonyms
Gastrointestinal NET, Gastroenteropancreatic Neuroendocrine Tumor, Digestive System NET, Gastroenteropancreatic NET, Gastrointestinal Neuroendocrine Tumor, Digestive System Well Differentiated Neuroendocrine Tumor, GEP-NET, Digestive System Neuroendocrine Tumor, Gastrointestinal System Neuroendocrine Tumor
Parent(s)
Digestive System Neuroendocrine Neoplasm
Children
L-Cell Glucagon-Like Peptide-Producing Neuroendocrine Tumor, VIP-Producing Neuroendocrine Tumor, Appendix Neuroendocrine Tumor, Colorectal Neuroendocrine Tumor, Gastric Neuroendocrine Tumor, Small Intestinal Neuroendocrine Tumor, Somatostatin-Producing Neuroendocrine Tumor, Gastrin-Producing Neuroendocrine Tumor, Esophageal Neuroendocrine Tumor, and Pancreatic 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 6. 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.