Department of Pediatrics and Pediatric Hematology and Oncology, Zentrum für Kinder- und Jugendmedizin, Klinikum Oldenburg gGmbH, Rahel-Straus-Strasse 10, 26133 Oldenburg, Germany.
Expert Rev Neurother. 2010 Apr;10(4):515-24. doi: 10.1586/ern.10.15.
Craniopharyngiomas are embryogenic malformations of the sellar region of low histological malignancy, thought to be derived from Rathke's pouch epithelium. With an overall incidence of 0.5-2.0 new cases per million of the population per year, 30-50% of all cases occur in childhood. Overall survival rates are high. However, quality of survival is substantially reduced in many survivors, owing to long-term sequelae, such as extreme obesity caused by hypothalamic tumor involvement, which occurs in up to 40% of all cases. The treatment of craniopharyngioma remains controversial. Radical surgical strategies are associated with poor outcome in craniopharyngioma with hypothalamic involvement.
颅咽管瘤是鞍区的胚胎性畸形,组织学恶性程度低,被认为来源于 Rathke 囊上皮。其年发病率为每百万人口中有 0.5-2.0 例新发病例,30-50%的病例发生在儿童期。总体生存率较高。然而,由于长期后遗症,许多幸存者的生存质量大大降低,如由于下丘脑肿瘤受累引起的极度肥胖,这种情况在所有病例中发生比例高达 40%。颅咽管瘤的治疗仍存在争议。对于下丘脑受累的颅咽管瘤,激进的手术策略与不良预后相关。