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

Appendix neuroendocrine tumors most frequently harbor alterations in SMAD4, KRAS, YES1, SPOP, and SMARCA4 [2].

Most Commonly Altered Genes in Appendix Neuroendocrine Tumor

KRAS Mutation, KRAS Exon 2 Mutation, KRAS Codon 12 Missense, YES1 Loss, and SPOP Y87C are the most common alterations in appendix neuroendocrine tumor [2].

Top Alterations in Appendix Neuroendocrine Tumor

Disease Details

Appendix Well Differentiated Endocrine Tumor, Appendix NET, Appendix Well Differentiated Endocrine Tumor/Carcinoma
Gastrointestinal Neuroendocrine Tumors
Appendix L-Cell Glucagon-Like Peptide-Producing Neuroendocrine Tumor
OncoTree Name
Well-Differentiated Neuroendocrine Tumor of the Appendix
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 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.