Chapter 39 - Adult craniopharyngiomas

Abstract

Craniopharyngiomas (CPs) are intracranial, extraaxial tumors of the sellar and parasellar region that frequently contain cystic components. CPs occur during childhood and in adulthood. Adult CPs have a peak manifestation during the 5th and 6th decades of life. Two histopathological subtypes, papillary and adamantinomatous, have been identified, and both are benign World Health Organization grade I lesions. Papillary CP is diagnosed almost exclusively in adults; in contrast, adamantinomatous CP is found in both children and adults. Clinical presentation varies depending on tumor size and location. Typical symptoms leading to diagnostic workup are vision disorders caused by irritation of the optic system and/or hormone disorders due to the tumor's affecting the hypothalamic region, the pituitary gland, and/or the pituitary stalk. Diagnostic workup includes cranial imaging (magnetic resonance imaging and computerized tomography), ophthalmological exam, and hormone evaluation. Currently, first-line therapy is surgical resection of the tumor. In case of complete surgical resection, long-term survival is favorable, with a 10-year survival rate of >90%. In case of incomplete surgical resection, however, tumor recurrence is common, and repeated surgical and nonsurgical therapies may become necessary, with radiation therapy considered second-line treatment. Quality of life is frequently limited by persisting hormone disorders and irritation of the hypothalamic region. With improved knowledge of cellular pathways and tumor mutations in CPs, new approaches have been described. The BRAF V600 mutation, which is found in papillary CPs, is a promising therapeutic target. However, pharmacotherapy and immunotherapy have not yet been established as standard therapy and are currently individualized approaches in patients for whom standard therapies have failed.