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Nat Rev Endocrinol. 2010 Nov;6(11):609-18. doi: 10.1038/nrendo.2010.168. Epub 2010 Sep 28.

Childhood craniopharyngioma--current concepts in diagnosis, therapy and follow-up.

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Department of Pediatrics and Pediatric Hematology and Oncology, Zentrum für Kinder- und Jugendmedizin, Klinikum Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, Germany.


Craniopharyngiomas have an overall incidence of 0.5-2.0 new cases per million of the population per year, and ∼30-50% of all cases represent childhood craniopharyngioma. These partly cystic embryogenic malformations of the sellar region are presumably derived from Rathke cleft epithelium. Many of the typical manifestations at primary diagnosis are nonspecific and include headache, visual impairment, polyuria and/or polydypsia, growth retardation and weight gain. Total resection is the treatment of choice in patients with favorable tumor localization, with the intention to maintain hypothalamic-pituitary and optical nerve functions. When the tumor localization is unfavorable, a limited resection followed by local irradiation is recommended. The overall survival rates are high (91-98%). High recurrence rates after complete resection and high progression rates after incomplete resection have been observed, although the risk of recurrence or progression is less after complete resection than partial resection. Irradiation of the tumor is protective and the appropriate time point of irradiation after incomplete resection is currently under investigation in a randomized trial. Long-term sequelae substantially reduce the quality of life of ∼50% of long-term survivors, notably extreme obesity owing to hypothalamic involvement.

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