NCI Definition: A malignant epithelial neoplasm arising from the appendix. The vast majority of the cases are adenocarcinomas. [1]

Appendix carcinomas most frequently harbor alterations in KRAS, TP53, GNAS, SMAD4, and APC [2].

Most Commonly Altered Genes in Appendix Carcinoma

KRAS Mutation, KRAS Exon 2 Mutation, KRAS Codon 12 Missense, GNAS Mutation, and KRAS G12D are the most common alterations in appendix carcinoma [2].

Top Alterations in Appendix Carcinoma

Disease Details

Carcinoma of the Appendix, Carcinoma of Appendix, Appendix Cancer
Malignant Appendix Neoplasm
Appendix Mixed Adenoneuroendocrine Carcinoma and Appendix Adenocarcinoma


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.