NCI Definition: A malignant epithelial neoplasm arising from the appendix. The vast majority of the cases are adenocarcinomas. 
Appendix carcinomas most frequently harbor alterations in KRAS, TP53, GNAS, SMAD4, and APC .
KRAS Mutation, KRAS Exon 2 Mutation, KRAS Codon 12 Missense, GNAS Mutation, and KRAS G12D are the most common alterations in appendix carcinoma .
There are 3 clinical trials for appendix carcinoma, of which 2 are open and 1 is completed or closed. Of the trials that contain appendix carcinoma as an inclusion criterion, 1 is phase 1 (1 open), 1 is phase 1/phase 2 (1 open), and 1 is phase 2 (0 open).
Oncos-102, bintrafusp alfa, and carboplatin are the most common interventions in appendix carcinoma 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.