Associated Genetic Biomarkers
NCI Definition: A malignant neoplasm arising from the glandular epithelium of the appendix with invasion of the appendiceal wall beyond the muscularis mucosa. The majority of cases are well differentiated adenocarcinomas with mucinous stroma formation. Clinically, it may present as an abdominal mass or with symptoms of acute appendicitis. Patients with chronic ulcerative colitis are at a higher risk in developing appendiceal adenocarcinomas compared to the general population. 
Appendix adenocarcinomas most frequently harbor alterations in KRAS, TP53, SMAD4, APC, and SOX9 .
KRAS Mutation, KRAS Exon 2 Mutation, KRAS Codon 12 Missense, KRAS G12D, and TP53 Mutation are the most common alterations in appendix adenocarcinoma .
There are 3 clinical trials for appendix adenocarcinoma, of which 3 are open and 0 are completed or closed. Of the trials that contain appendix adenocarcinoma as an inclusion criterion, 2 are phase 1 (2 open) and 1 is phase 2 (1 open).
Deficient, MLH1, and MLH3 are the most frequent gene inclusion criteria for appendix adenocarcinoma clinical trials .
Bevacizumab, mk-8353, and atezolizumab are the most common interventions in appendix adenocarcinoma 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 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.