Update on management of craniopharyngiomas.

Abstract

Craniopharyngiomas are locally-aggressive tumors arising along the hypothalamic-pituitary axis. Treatment is nuanced as a result of their proximity and adherence to vital neurovascular structures and responsiveness to surgery, radiation and, in some cases, chemotherapy. We reviewed the literature discussing the current state of knowledge regarding craniopharyngioma biology and therapy. Recent advances in endoscopic endonasal surgery (EEA) have made surgery a safer and more effective option. While cure may be achieved with gross total resection (GTR), when felt to be too risky, a subtotal resection followed by radiation is often a more prudent strategy, particularly in children with hypothalamic invasion. Data on long-term outcome are mostly derived from older studies in which a craniotomy, rather than EEA, was performed. Long-term EEA outcome studies are lacking. Enhanced knowledge of the biological basis of papillary CPs has led to novel medical treatments for BRAFv600E variants that appear to be effective. Endoscopic technology has improved surgical results for craniopharyngiomas and expanded the indications for the transsphenoidal approach. The goal of CP surgery goal is maximal safe resection to achieve cure, but subtotal resection and radiation may be equally effective. Early diagnosis of specific variants will facilitate enrollment in promising medical trials.