Craniopharyngioma is a term used to denote two separate entities (adamantinomatous craniopharyngiomas and papillary craniopharyngiomas), both relatively benign (WHO grade 1) neoplasms that typically arise in the sellar/suprasellar region.
Terminology
Until the 5th Edition (2021) of the WHO classification of CNS tumors, adamantinomatous craniopharyngiomas and papillary craniopharyngiomas were considered subtypes of craniopharyngiomas 1.
Increasing molecular evidence has demonstrated that they have distinct and mutually exclusive mutations of CTNNB1 (adamantinomatous) and BRAF (papillary).
As such, they are now considered separate entities and will be discussed separately.
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