Department of Pediatrics and Pediatric Hematology and Oncology, Zentrum für Kinder- und Jugendmedizin, Klinikum Oldenburg, Rahel-Straus-Strasse 10, 26133 Oldenburg, Germany.
Nat Rev Endocrinol. 2010 Nov;6(11):609-18. doi: 10.1038/nrendo.2010.168. Epub 2010 Sep 28.
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.
颅咽管瘤的年发病率为每百万人口中 0.5-2.0 例，约 30-50%的病例为儿童颅咽管瘤。这些部分囊性的胚胎发育不良的鞍区肿瘤，推测来源于 Rathke 裂上皮。许多初次诊断时的典型表现是非特异性的，包括头痛、视力损害、多尿和/或多饮、生长发育迟缓以及体重增加。对于具有良好肿瘤定位的患者，全切除是首选治疗方法，目的是维持下丘脑-垂体和视神经的功能。当肿瘤定位不佳时，建议进行有限的切除，然后进行局部照射。总体生存率较高（91-98%）。尽管全切除比部分切除的复发风险小，但完全切除后仍有较高的复发率，不完全切除后有较高的进展率。肿瘤照射具有保护作用，目前正在一项随机试验中研究不完全切除后照射的适当时间点。长期后遗症显著降低了约 50%的长期幸存者的生活质量，尤其是由于下丘脑受累导致的极度肥胖。
