案例#1
一名 2 岁女孩,出现恶心、呕吐以及头颅增大持续 2-3 周。查体发现头颅增大和视力低下(视敏度 20/200)
案例#2
16 岁女孩表现为原发性闭经、泌乳和轻度头痛。眼科检查示:右眼视力丧失(视敏度 20/40)
其他表现
约 50% 的患者表现为急性颅内压增高的症状和体征(头痛、恶心、呕吐、感觉减退、复视)以及急性视力减退,需要急诊手术治疗。[2]Van Effenterre R, Boch AL. Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg. 2002;97:3-11.http://www.ncbi.nlm.nih.gov/pubmed/12134929?tool=bestpractice.com[3]Carmel PW, Antunes JL, Chang CH. Craniopharyngiomas in children. Neurosurgery. 1982;2:382-389.http://www.ncbi.nlm.nih.gov/pubmed/7133354?tool=bestpractice.com[4]Karavitaki N, Cudlip S, Adams CB, et al. Craniopharyngiomas. Endocr Rev. 2006;27:371-397.http://edrv.endojournals.org/content/27/4/371.longhttp://www.ncbi.nlm.nih.gov/pubmed/16543382?tool=bestpractice.com 尽管视力障碍通常不是唯一的症状表现,但是术前出现视力障碍通常见于至少 75% 的患者。[2]Van Effenterre R, Boch AL. Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg. 2002;97:3-11.http://www.ncbi.nlm.nih.gov/pubmed/12134929?tool=bestpractice.com[4]Karavitaki N, Cudlip S, Adams CB, et al. Craniopharyngiomas. Endocr Rev. 2006;27:371-397.http://edrv.endojournals.org/content/27/4/371.longhttp://www.ncbi.nlm.nih.gov/pubmed/16543382?tool=bestpractice.com[5]Baskin DS, Wilson CB. Surgical management of craniopharyngiomas. A review of 74 cases. J Neurosurg. 1986;65:22-27.http://www.ncbi.nlm.nih.gov/pubmed/3712025?tool=bestpractice.com[6]Defoort-Dhellemmes S, Moritz F, Bouacha I, et al. Craniopharyngioma: ophthalmological aspects at diagnosis. J Pediatr Endocrinol Metab. 2006;19(suppl 1):321-324.http://www.ncbi.nlm.nih.gov/pubmed/16700306?tool=bestpractice.com 就诊时,约 30%-80% 的患者出现内分泌障碍,例如尿崩症、生长发育障碍,成人可表现为性功能障碍(勃起障碍、闭经以及泌乳等)。[2]Van Effenterre R, Boch AL. Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg. 2002;97:3-11.http://www.ncbi.nlm.nih.gov/pubmed/12134929?tool=bestpractice.com[4]Karavitaki N, Cudlip S, Adams CB, et al. Craniopharyngiomas. Endocr Rev. 2006;27:371-397.http://edrv.endojournals.org/content/27/4/371.longhttp://www.ncbi.nlm.nih.gov/pubmed/16543382?tool=bestpractice.com[7]Bunin GR, Surawicz TS, Witman PA, et al. The descriptive epidemiology of craniopharyngiomas. J Neurosurg. 1998;89:547-551.http://www.ncbi.nlm.nih.gov/pubmed/9761047?tool=bestpractice.com[8]Halac I, Zimmerman D. Endocrine manifestations of craniopharyngioma. Childs Nerv Syst. 2005;21:640-648.http://www.ncbi.nlm.nih.gov/pubmed/16047216?tool=bestpractice.com[9]Hopper N, Albanese A, Ghirardello S, et al. The preoperative endocrine assessment of craniopharyngiomas. J Pediatr Endocrinol Metab. 2006;19(suppl 1):325-327.http://www.ncbi.nlm.nih.gov/pubmed/16700307?tool=bestpractice.com 然而,单纯内分泌症状在青少年及成人更为常见。[4]Karavitaki N, Cudlip S, Adams CB, et al. Craniopharyngiomas. Endocr Rev. 2006;27:371-397.http://edrv.endojournals.org/content/27/4/371.longhttp://www.ncbi.nlm.nih.gov/pubmed/16543382?tool=bestpractice.com[8]Halac I, Zimmerman D. Endocrine manifestations of craniopharyngioma. Childs Nerv Syst. 2005;21:640-648.http://www.ncbi.nlm.nih.gov/pubmed/16047216?tool=bestpractice.com[10]Dhellemmes P, Vinchon M. Radical resection for craniopharyngiomas in children: surgical technique and clinical results. J Pediatr Endocrinol Metab. 2006;19(suppl 1):329-335.http://www.ncbi.nlm.nih.gov/pubmed/16700308?tool=bestpractice.com 颅咽管瘤最常见的表现是上述症状混合出现,包括视力丧失、颅内压升高以及内分泌障碍。[2]Van Effenterre R, Boch AL. Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg. 2002;97:3-11.http://www.ncbi.nlm.nih.gov/pubmed/12134929?tool=bestpractice.com[4]Karavitaki N, Cudlip S, Adams CB, et al. Craniopharyngiomas. Endocr Rev. 2006;27:371-397.http://edrv.endojournals.org/content/27/4/371.longhttp://www.ncbi.nlm.nih.gov/pubmed/16543382?tool=bestpractice.com[11]Garre ML, Cama A. Craniopharyngioma: modern concepts in pathogenesis and treatment. Curr Opin Pediatr. 2007;19:471-479.http://www.ncbi.nlm.nih.gov/pubmed/17630614?tool=bestpractice.com[12]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