Diagnosis and treatment of craniopharyngioma

Craniopharyngiomas are rare embryonal malformations. Quality of life especially in case of hypothalamic tumor involvement is impaired by hypothalamic obesity, fatigue, and psychosocial deficits. Patients with hypothalamic involvement present with reduced overall survival rates, whereas overall and progression-free survival are not associated with surgical degree of resection. Radiation therapy is effective in prevention and treatment of progression/recurrence. Preliminary experiences with proton beam therapy are promising, offering a more protective radio oncological treatment. Novel insights in molecular pathogenesis of craniopharyngiomas have offered the possibility of targeted therapy and testing treatment in animal models. Treatment options for hypothalamic syndrome are limited. Accordingly, hypothalamus-sparing strategies are recommended. This review summaries novel insights in diagnostics and treatment of craniopharyngioma based on reports published since 2015.