Craniopharyngioma

Current management for craniopharyngioma, a low-grade developmental neoplasm of the sellar region, is complicated by the tumor’s proximity to critical neuroanatomical structures, including the optic chiasm and hypothalamus. Recommended preoperative investigations include MRI with and without gadolinium infusion, a neuro-ophthalmologic examination and a complete endocrine evaluation. Strict management of fluids and electrolytes, the judicious use of desmopressin (DDAVP), and the provision of ‘stress’ doses of gluocorticoids will help guide the patient through the perioperative period. Although radical resection has a role as the sole therapeutic modality in some instances, the majority of patients can be appropriately managed by a subtotal resection followed by adjuvant external beam radiotherapy to the involved field using three dimensional treatment planning. The use of external ventricular drains may obviate the requirement for an indwelling cerebrospinal fluid shunt device. Patients with recurrent or progressive disease, in particular those with cystic tumors, may benefit from other therapies, including stereotactic radiosurgery (gamma knife) or intracavitary irradiation. Currently, stereotactic radiotherapy, intracavitary bleomycin, and systemic chemotherapy remain investigational treatment modalities for this challenging disorder.