Childhood craniopharyngioma

Mueller, H. L. and Gebhardt, U. and Kortmann, R-D. and Pohl, F. and Pietsch, T. and Warmuth-Metz, M. and Kaatsch, P. and Faldum, A. and Zwiener, I. and Calaminus, G. and Kolb, R. and Wiegand, C. and Soerensen, N. (2008) Childhood craniopharyngioma. MONATSSCHRIFT KINDERHEILKUNDE, 156 (12). pp. 1194-1200. ISSN 0026-9298, 1433-0474

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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.

Item Type: Article
Uncontrolled Keywords: BODY-MASS INDEX; INCREASED DAYTIME SLEEPINESS; QUALITY-OF-LIFE; FUNCTIONAL-CAPACITY; SURGICAL-TREATMENT; OBESITY; CHILDREN; HYPERPHAGIA; DIAGNOSIS; Craniopharyngioma; Hypothalamus; Obesity; Quality of life; Irradiation
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Strahlentherapie
Depositing User: Dr. Gernot Deinzer
Date Deposited: 16 Oct 2020 12:05
Last Modified: 16 Oct 2020 12:05
URI: https://pred.uni-regensburg.de/id/eprint/30006

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