Treatment of Cystic Craniopharyngiomas: An Update.

In spite of the significant technical and technological progress in neurosurgery and the continuous discoveries by the basic research, adamantinomatous craniopharyngioma remains a significant clinical challenge. Actually, the huge size of the tumor, its multiple cystic components, the encasement of Willis' circle and optic pathways, and the invasion of the hypothalamus often prevent its safe surgical resection. Moreover, the local aggressiveness of the tumor accounts for a high risk of recurrence even after a gross total resection. For these reasons, more and more efforts are being dedicated to enhance the knowledge about AC and improve the tools for its treatment.This paper is dedicated to the most recent advances concerning the AC management. Promising, new insights come for the basic research, thanks to the updates on the role of the WNT-β-catenin pathway (important for the tumor genesis and progression, not yet developed enough for a safe target therapy in children but useful for determining the prognosis) and the inflammatory mediators (widely overexpressed, especially by the cyst of the tumor, and for which target therapies are being developed). Moreover, further factors and pathways are under investigation.Also the development of new treatment strategies accounts for the improvement of the prognosis and the quality of life of AC patients. The enhancement of the experience with the endoscopic techniques (both transsphenoidal and transventricular approaches) actually allows to perform a less invasive but effective surgery that can be coupled with new modalities of radiation therapy aiming at obtaining a reliable control of the disease and protecting the endocrinological, ophthalmological, and neurological functions. A special mention is finally deserved by the techniques specifically designed for the intracystic therapy (as cyst fenestration alone or in combination with administration of radionuclides or bleomycin or interferon-α) that are here analyzed together with the aforementioned advances.