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Diagnosis and management of craniopharyngiomas in the era of genomics and targeted therapy

Authors :
Fred G. Barker
Sandro Santagata
Daniel P. Cahill
Megan R. D'Andrea
Priscilla K. Brastianos
Juan Carlos Martinez-Gutierrez
Source :
Neurosurgical Focus. 41:E2
Publication Year :
2016
Publisher :
Journal of Neurosurgery Publishing Group (JNSPG), 2016.

Abstract

Craniopharyngiomas are rare intracranial neoplasms that pose clinical challenges due to their location adjacent to vital structures. The authors have previously shown high mutation rates of BRAF V600E in papillary craniopharyngioma and of CTNNB1 in adamantinomatous craniopharyngioma. These activating driver mutations are potential therapeutic targets, and the authors have recently reported a significant response to BRAF/MEK inhibition in a patient with multiply recurrent PCP. As these targetable mutations warrant prospective research, the authors will be conducting a national National Cancer Institute–sponsored multicenter clinical trial to investigate BRAF/MEK inhibition in the treatment of craniopharyngioma. In this new era of genomic discovery, the treatment paradigm of craniopharyngioma is likely to change.

Details

ISSN :
10920684
Volume :
41
Database :
OpenAIRE
Journal :
Neurosurgical Focus
Accession number :
edsair.doi.dedup.....1030a9e660376200e750b90a72a08d9b