The Medical Therapy of Craniopharyngiomas: The Way Ahead

The Journal of Clinical Endocrinology and Metabolism
Krystallenia I AlexandrakiAshley B Grossman

Abstract

Craniopharyngiomas, which are categorized as adamantinomatous (ACPs) or papillary (PCPs), have traditionally been treated with surgery and/or radiotherapy, although when the tumors progress or recur, therapeutic possibilities are very limited. Following recent advances in their molecular pathogenesis, new medical therapeutic options have emerged. The search strategy that we selected to identify the appropriate evidence involved the following medical subject headings (MeSH) terms: ("Craniopharyngioma" [MeSH] AND "Craniopharyngioma/drug therapy" [MeSH]) NOT ("review" [Publication Type] OR "review literature as topic" [MeSH Terms] OR "review" [All Fields]) AND ("2009/05/01" [PDat]: "2019/04/28" [PDat]). Mutations of β-catenin causing Wnt activation with alterations of the MEK/ERK pathway are encountered in the great majority of patients with ACPs; specific alterations also stratify patients to a more aggressive behavior. In most PCPs there is primary activation of the Ras/Raf/MEK/ERK pathway secondary to BRAF-V600E mutations. BRAF inhibitors, such as dabrafenib or vemurafenib, either alone or in combination with the MEK inhibitors trametinib and cobimetinib, have been administered to patients with PCPs producing clinically useful ...Continue Reading

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Citations

Sep 24, 2020·Endocrine Pathology·Noriaki FukuharaHiroshi Nishioka
May 22, 2021·Developmental Medicine and Child Neurology·Tim Cheetham, Claire Wood
Oct 8, 2021·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·M Mazzuia GuimarãesH Matushita
Mar 4, 2020·Neuroendocrinology·Juan Pedro Martinez-Barbera, Cynthia Lilian Andoniadou
Mar 11, 2020·Neuroendocrinology·Emilija Manojlovic-GacicOlivera Casar-Borota
Nov 23, 2021·Journal of Neuro-oncology·Fraser Henderson, Theodore H Schwartz

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