Overview

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, SMAD4, APC, and SOX9 [2].

Most Commonly Altered Genes in Appendix Carcinoma

KRAS Mutation, KRAS Exon 2 Mutation, KRAS Codon 12 Missense, KRAS G12D, and TP53 Mutation are the most common alterations in appendix carcinoma [2].

Top Alterations in Appendix Carcinoma

Significant Genes in Appendix Carcinoma

MLH1 +

MLH3 +

MSH2 +

MSH3 +

MSH6 +

PMS1 +

PMS2 +

Disease Details

Synonyms
Appendix Cancer, Carcinoma of the Appendix, Carcinoma of Appendix
Parent(s)
Malignant Appendix Neoplasm
Children
Appendix Adenocarcinoma and Appendix Mixed Adenoneuroendocrine Carcinoma

References

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.