Small Intestinal Neuroendocrine Tumor
Associated Genetic Biomarkers
NCI Definition: A well differentiated, low or intermediate grade tumor with neuroendocrine differentiation that arises from the small intestine. 
Small intestinal neuroendocrine tumors most frequently harbor alterations in CDKN1B, BCOR, POLQ, MEN1, and EP300 .
BCOR Mutation, CDKN1fs, POLQ Mutation, SETD2 Mutation, and CDKN1B Mutation are the most common alterations in small intestinal neuroendocrine tumor .
Of the biomarker-directed therapies for small intestinal neuroendocrine tumor, 1 is FDA-approved in at least one setting and 0 have NCCN guidelines in at least one setting .
There are 8 clinical trials for small intestinal neuroendocrine tumor, of which 6 are open and 2 are completed or closed. Of the trials that contain small intestinal neuroendocrine tumor as an inclusion criterion, 2 are phase 1/phase 2 (1 open) and 6 are phase 2 (5 open).
SSTR2, SSTR1, and SSTR3 are the most frequent gene inclusion criteria for small intestinal neuroendocrine tumor clinical trials .
Pembrolizumab, 177lu-dota-octreotate, and 177lu-opsc001 are the most common interventions in small intestinal neuroendocrine tumor clinical trials.
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.