Craniopharyngiomas: an update

Craniopharyngiomas have been challenging practitioners for over 100 years. These benign cystic tumors are thought to be a part of a continuum of ectodermal cystic lesions found in the suprasellar region. They are rare tumors found in both the adult and pediatric populations. Patients most often present with findings associated with ventricular outflow obstruction causing hydrocephalus, optic chiasm compression resulting in visual dysfunction or hypothalamic/pituitary compression leading to endocrinopathy. The mainstay of treatment involves a combination of radical or limited surgery followed by radiotherapy. Other adjunctive strategies include cyst fenestration, Ommaya catheter implantation and brachytherapy. Patients in general have high long-term survival but can have significant tumor- and treatment-associated morbidity requiring a lifetime of medical and psychological management.