Childhood craniopharyngioma.: Current concepts in diagnostics and therapy

Craniopharyngiomas are benign, partly cystic, epithelial tumors of the sellar region. Between, 30% and 50% of the cases manifest during childhood and adolescence and represent 5-10% of all cases of intracranial tumors during childhood. The therapy of choice in patients with favourable tumor location is total microscopic resection while maintaining the visual and hypothalamic-pituitary function. In patients with unfavourable tumor location,this therapeutic strategy is subject to controversy, and consideration should be given to limited resection followed by radiation therapy. The overall survival rates are high. However, the quality of life (QoL) is substantially reduced in many survivors due to neuropsychological, ophthalmologic,and endocrine sequelae. Extreme obesity caused by eating disorders of hypothalamic origin has a major negative impact on QoL. In a cross-sectional multicenter study (n=138), QoL was not related to intended or realized degree of surgical resection. A multicenter study (Kraniopharyngeom 2000; English-language study protocol available at: www.kranio-pharyngeom.com) was initiated to analyze prospectively the survival rate and QoL in newly diagnosed patients with childhood craniopharyngioma in relation to different therapeutic strategies. Based on the high rate of compliance of participating centers in Germany, Austria, and Switzerland,we hope to substantially improve the extent of epidemiologic registration of this rare disease in the near future.