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]

Significant Genes in Craniopharyngioma

MYCN +

Disease Details

Synonyms
CRANIOPHARYNGIOMA, BENIGN, Rathke's Pouch Tumor, Cystoma, Tumor of Rathke's Pouch, Neoplasm of Rathke's Pouch, Rathke Pouch Neoplasm, Craniopharyngioma (WHO Grade I), Rathke Pouch Tumor, Rathke's Pouch Neoplasm
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 8. 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.