NCI Definition: A well differentiated, low or intermediate grade tumor with neuroendocrine differentiation that arises from the small intestine. [1]

Small intestinal neuroendocrine tumors most frequently harbor alterations in CDKN1B and BCOR [2].

Most Commonly Altered Genes in Small Intestinal Neuroendocrine Tumor

CDKN1B Mutation and BCOR Mutation are the most common alterations in small intestinal neuroendocrine tumor [2].

Top Alterations in Small Intestinal Neuroendocrine Tumor

Significant Genes in Small Intestinal Neuroendocrine Tumor

MLH1 +

MLH3 +

MSH2 +

MSH3 +

MSH6 +

PMS1 +

PMS2 +

Disease Details

Small Intestinal NET, Small Intestinal Well Differentiated Endocrine Tumor, Small intestine neuroendocrine carcinoma, Small Intestinal Well Differentiated Endocrine Tumor/Carcinoma
Small Intestinal Neuroendocrine Neoplasm
Small Intestinal VIP-Producing Neuroendocrine Tumor, Duodenal Neuroendocrine Tumor, Jejunal Somatostatin-Producing Neuroendocrine Tumor, and Small Intestinal L-Cell Glucagon-Like Peptide-Producing Neuroendocrine Tumor
OncoTree Name
Small Bowel Well-Differentiated Neuroendocrine Tumor
OncoTree Code


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.