摘要
目的总结儿童库欣病的临床特点和治疗经验。方法回顾性分析27例年龄小于14岁儿童库欣病的临床资料。男15例,女12例,平均发病年龄10.7岁。术前影像学检查显示:微腺瘤20例,大腺瘤4例,未见异常3例。均行经蝶垂体微腺瘤切除加瘤周垂体大部切除术。结果肿瘤全切除26例,残留1例。术后病理证实垂体促肾上腺皮质激素(ACTH)腺瘤16例,垂体ACTH细胞增生3例,未发现腺瘤或增生7例。术后内分泌治愈率、缓解率和无效率分别是59.3%、7.4%和33.3%。平均随访30.4个月(6～120个月),复发4例(14.8%)。术后因复发或症状未缓解再次经蝶手术4例,垂体普通放疗5例,垂体伽玛刀放疗2例,肾上腺切除7例;出现Nelson综合征2例。结论经蝶垂体微腺瘤切除加瘤周垂体大部切除术是治疗儿童库欣病的首选和有效治疗方法,垂体放疗和肾上腺切除是二线治疗方法。
Objectives To summarize the clinical characteristics and treatment experiences of Cushing disease in children.Methods The clinical data of 27 children,including 15 males and 12 females,under 14 years old with Cushing disease was analyzed retrospectively.All the patients whose mean age-at onset was 10.7 years underwent transsphenoidal resection of pituitary adenoma together with subtotal peritumor pituitary tissue resection.The tumors were examined by imaging techniques preoperatively,including microadenomas in 20 cases,macroadenomas in 4,and negative in 3.Results Total tumor resection was achieved in 26 patients and residual tumor left in 1.The diagnosis was established by postoperative pathological analysis,including ACTH-secreting pituitary adenoma in 16 cases,hyperplasia of pituitary ACTH cells in 3,no adenoma or hyperplasia in 7.The cure rate,remission rate and ineffectiveness rate due to endocrine therapy after surgery were 59.3%,7.4% and 33.3%,respectively.Cushing disease reoccurred in 4 patients（14.8%）during the follow up period of 30.4 months,ranged from 6 to 120 months.The patients with recurrence or no remission received retreatments including transsphenoidal surgery again in 4 patients,pituitary radiotherapy in 5,pituitary gamma knife in 2 and bilateral adrenalectomy in 7.Nelson＇s syndrome appeared in 2 cases.Conclusions Transsphenoidal resection of pituitary adenoma together with peritumor pituitary tissue is the first choice in the treatment of Cushing disease in children and pituitary radiotherapy or adrenalectomy is the second line therapy.
出处
《中国微侵袭神经外科杂志》
CAS
北大核心
2010年第8期352-354,共3页
Chinese Journal of Minimally Invasive Neurosurgery
关键词
库欣病
促肾上腺皮质激素
儿童
经蝶手术
cushing disease
adrenocorticotrophic hormone
child
transsphenoidal surgery
