Abstract
Craniopharyngiomas are benign, partly cystic embryogenic malformations of the sellar region, presumably derived from Rathke pouch epithelium. With an overall incidence of 0.5-2 new cases per million population per year, 30-50% of all cases represent childhood craniopharyngioma. Typical manifestations at primary diagnosis are headache, visual disturbance, polyuria/polydipsia, growth retardation, ...
Abstract
Craniopharyngiomas are benign, partly cystic embryogenic malformations of the sellar region, presumably derived from Rathke pouch epithelium. With an overall incidence of 0.5-2 new cases per million population per year, 30-50% of all cases represent childhood craniopharyngioma. Typical manifestations at primary diagnosis are headache, visual disturbance, polyuria/polydipsia, growth retardation, and weight gain. The treatment of choice in patients with favorable tumor localization is total resection with the intention to maintain visual and hypothalamic-pituitary function. In patients with unfavorable tumor localization (hypothalamic involvement), a limited resection followed by radiation therapy is recommended. The appropriate time point for irradiation after incomplete resection is currently being investigated in a randomized trial (KRANIOPHARYNGEOM 2007). The overall survival rates are high (92%). However, quality of life is substantially reduced in many survivors due to sequelae. In particular, extreme obesity due to hypothalamic involvement has a major negative impact on quality of life.
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