NCI Definition: A low or intermediate grade well differentiated tumor with neuroendocrine differentiation that arises from the pancreas. According to the presence or absence of clinical syndromes that result from hormone hypersecretion, pancreatic neuroendocrine tumors are classified either as functional or nonfunctional. [1]

Pancreatic neuroendocrine tumors most frequently harbor alterations in MEN1, ATRX, TP53, CDKN2A, and TSC2 [2].

Most Commonly Altered Genes in Pancreatic Neuroendocrine Tumor

MEN1 Mutation, ATRX Mutation, TP53 Mutation, CDKN2A Loss, and TSC2 Mutation are the most common alterations in pancreatic neuroendocrine tumor [2].

Top Alterations in Pancreatic Neuroendocrine Tumor

Significant Genes in Pancreatic Neuroendocrine Tumor

MLH1 +

MLH3 +

MSH2 +

MSH3 +

MSH6 +

PMS1 +

PMS2 +

Disease Details

Pancreas neuroendocrine tumor (pNET), Islet Cell Tumor, PanNET, Well Differentiated Pancreatic Endocrine Neoplasm, Islet cell tumors - pancreas, Well Differentiated Pancreatic Endocrine Tumor, Pancreatic NET, Islet cell tumors of the pancreas
Pancreatic Neuroendocrine Neoplasm
Functional Pancreatic Neuroendocrine Tumor, Non-functioning Pancreatic Endocrine Tumor, Pancreatic Insulin-Producing Neuroendocrine Tumor, Pancreatic Delta Cell Neuroendocrine Tumor, Pancreatic Gastrin-Producing Neuroendocrine Tumor, and Pancreatic VIP-Producing Neuroendocrine Tumor


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.