Management of Craniopharyngiomas in the Era of Molecular Oncological Therapies: Not a Panacea

Abstract

Papillary craniopharyngiomas (PCPs) in the pediatric population are vanishingly rare (see brief review by Borrill et al. [ 1]); the vast majority in this age group being of the adamantinomatous (ACP) subtype. As alluded to by the authors [ 2], the major difference between these 2 histologies are usually mutually exclusive mutations in BRAF (specifically V600E, activating signaling via the MEK/MAPK pathway) and CTNNB1 (causing hyperactivation of the WNT signaling pathway) respectively [ 3]. Both of these pathways have been implicated in tumorigenesis of a vast spectrum of benign and malignant tumors. It is worth noting, however, that coexistence of both BRAF V600E and CTNNB1 mutations have been described in some ACPs [ 4]. Unfortunately in this case it was not possible to reanalyze the original tissue specimens obtained in childhood to elucidate whether this patient had a PCP or mixed ACP/PCP from the start presenting in childhood (more likely), or an initial ACP followed by a metachronous PCP arising later in adulthood. Transformation of an ACP to a PCP has never been described.