Craniopharyngioma

Craniopharyngiomas are rare benign squamous epithelial tumors arising along the path of the craniopharyngeal duct. Adamantiomatous craniopharyngioma and papillary craniopharyngioma are now considered two distinct tumor types. Adamantiomatous craniopharyngioma has a bimodal age distribution, with the incidence in childhood onset at 5–14 years and adult-onset at 50–74 years. Papillary craniopharyngioma almost exclusively occurs in adults. Clinical manifestations are often related to increased intracranial pressure, visual disturbances, and hypothalamic/pituitary deficiencies. Surgical resection is the treatment of choice for tumors with favorable locations. Limited-hypothalamic sparing surgery followed by radiation is the treatment of choice for tumors with an unfavorable location. Patients with a gross total resection may be safely observed. Radiation treatment is often indicated in patients with subtotal resections. Radiosurgery may be used for patients with small residual tumors and away from the optic apparatus. Radiation treatment is highly effective with a high local control rate. The overall survival is quite favorable. However, there is notable risk for late-onset morbidities, including hypothalamic–pituitary dysfunction, impairment of quality of life, neurocognition, and/or psychosocial functioning. Multidisciplinary care is critical for optimal management of craniopharyngiomas and treatment morbidities.