Overview

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) [1]

Craniopharyngiomas most frequently harbor alterations in CTNNB1 [2].

Most Commonly Altered Genes in Craniopharyngioma

CTNNB1 Mutation are the most common alterations in craniopharyngioma [2].

Top Alterations in Craniopharyngioma

Significant Genes in Craniopharyngioma

MYCN +

Disease Details

Synonyms
Neoplasm of Rathke's Pouch, Rathke Pouch Tumor, Rathke Pouch Neoplasm, CRANIOPHARYNGIOMA, BENIGN, Cystoma, Rathke's Pouch Tumor, Craniopharyngioma (WHO Grade I), Rathke's Pouch Neoplasm, Tumor of Rathke's Pouch
Parent(s)
Squamous Cell Neoplasm
Children
Papillary Craniopharyngioma and Adamantinomatous Craniopharyngioma

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.