中枢神经系统的良性轴外非胶质细胞上皮性肿瘤,儿童及成人均可发生。颅咽管瘤最常起源于鞍区或鞍上区。虽然该病可发生于任何年龄,但据报道,其发病年龄呈双峰分布,分别在儿童的 5-14 岁、成人的 50-70 岁。[16]Haupt R, Magnani C, Pavanello M, et al. Epidemiological aspects of craniopharyngioma. J Pediatr Endocrinol Metab. 2006;19(suppl 1):289-293.
http://www.ncbi.nlm.nih.gov/pubmed/16700303?tool=bestpractice.com
[17]Nielsen EH, Feldt-Rasmussen U, Poulsgaard L, et al. Incidence of craniopharyngioma in Denmark (n = 189) and estimated world incidence of craniopharyngioma in children and adults. J Neurooncol. 2011 Sep;104(3):755-63.
http://www.ncbi.nlm.nih.gov/pubmed/21336771?tool=bestpractice.com
[18]Zacharia BE, Bruce SS, Goldstein H, et al. Incidence, treatment and survival of patients with craniopharyngioma in the surveillance, epidemiology and end results program. Neuro Oncol. 2012 Aug;14(8):1070-8.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3408265
http://www.ncbi.nlm.nih.gov/pubmed/22735773?tool=bestpractice.com
[19]Lithgow K, Pohl U, Karavitaki N. Craniopharyngiomas. In: Feingold KR, Anawalt B, Boyce A, et al., eds. Endotext. South Dartmouth, MA: MDText.com, Inc.; 2019.
https://www.ncbi.nlm.nih.gov/books/NBK538819
http://www.ncbi.nlm.nih.gov/pubmed/30888745?tool=bestpractice.com
颅咽管瘤可以引起包括视力丧失、颅内压增高症状在内的占位效应症状。[20]Erfurth EM. Endocrine aspects and sequel in patients with craniopharyngioma. J Pediatr Endocrinol Metab. 2015;28:19-26.
http://www.ncbi.nlm.nih.gov/pubmed/25514328?tool=bestpractice.com
垂体功能障碍常见;儿童可能表现为生长不足,成人表现为尿崩症及性功能障碍。[21]Müller HL. Childhood craniopharyngioma. Pituitary. 2013;16:56-67.
http://www.ncbi.nlm.nih.gov/pubmed/22678820?tool=bestpractice.com
诊断性评估包括头颅 MRI、计算机体层成像和全面的内分泌功能评估。首要治疗是手术切除,对特定病例辅以术后放疗。[22]Karavitaki N, Cudlip S, Adams CB, et al. Craniopharyngiomas. Endocr Rev. 2006 Jun;27(4):371-97.
https://academic.oup.com/edrv/article/27/4/371/2355225
http://www.ncbi.nlm.nih.gov/pubmed/16543382?tool=bestpractice.com
[21]Müller HL. Childhood craniopharyngioma. Pituitary. 2013;16:56-67.
http://www.ncbi.nlm.nih.gov/pubmed/22678820?tool=bestpractice.com