NCI Definition: A benign, partly cystic, epithelial tumor of the sellar region, presumably derived from Rathke pouch epithelium. It affects mainly children and young adults. There are two clinicopathological forms: adamantinomatous craniopharyngioma and papillary craniopharyngioma. The most significant factor associated with recurrence is the extent of surgical resection, with lesions greater than 5 cm in diameter carrying a markedly worse prognosis. (Adapted from WHO) 
Craniopharyngiomas most frequently harbor alterations in CTNNB1, BRAF, TSC2, NF2, and KMT2D .
CTNNB1 Mutation, CTNNB1 S37F, BRAF V600E, BRAF Mutation, and BRAF Exon 15 Mutation are the most common alterations in craniopharyngioma .
There are 3 clinical trials for craniopharyngioma, of which 3 are open and 0 are completed or closed. Of the trials that contain craniopharyngioma as an inclusion criterion, 1 is early phase 1 (1 open), 1 is phase 1 (1 open), and 1 is no phase specified (1 open).
Pritumumab, standard of care, and tocilizumab are the most common interventions in craniopharyngioma clinical trials.
Significant Genes in Craniopharyngioma
MYCN is an inclusion eligibility criterion in 1 clinical trial for craniopharyngioma, of which 1 is open and 0 are closed. Of the trial that contains MYCN status and craniopharyngioma as inclusion criteria, 1 is no phase specified (1 open) .
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.