儿童及青少年垂体促肾上腺皮质激素腺瘤的临床特点及手术治疗效果

Surgical treatment of pituitary adrenocorticotropin-secreting adenoma in childhood and adolescence

目的探讨儿童及青少年垂体促肾上腺皮质激素腺瘤的临床特点及手术治疗效果。方法回顾性分析1987年12月至2014年12月北京协和医院收治的50例儿童和青少年垂体ACTH腺瘤患者的临床特点、内分泌学检查、影像学检查、肿瘤分型、手术入路、术后并发症及随访结果。结果 50例中男21例(42.0%),女29例(58.0%),年龄10~18岁,平均发病年龄15.5岁,平均病程45.5个月。术前影像学检查显示:微腺瘤40例(80.0%),大腺瘤10例(20.0%);侵袭性腺瘤4例(8.0%);均行经鼻蝶窦入路手术。肿瘤全切除48例(96.0%),次全切除2例(4.0%);随访时间1年,其中44例(88.0%)获得完整随访,7例(15.9%)在术后1年内复发或未缓解再次接受手术治疗。结论经鼻蝶入路手术是儿童及青少年垂体促肾上腺皮质激素腺瘤的首选和有效治疗方法,治疗效果良好。该病具有复发倾向,需要定期复查垂体MRI及垂体内分泌激素,复发者可行再次手术或放射治疗。

Objective The study aims to investigate the clinical characteristics and treatment of Cushing disease of childhood and adolescence. Methods In the study the data of 50 cases of pituitary adrenocorticotropin- secreting adenomas of childhood and adolescence, admitted to Peking Union Medical College Hospital from December 1997 to December 2014, were analyzed retrospectively, including the clinical manifestatiom, hormone secretions, images, pathological types, surgical complications and follow-up results. Results A total of 21 （42. 0% ） males and 29 females （58.0%） were included, whose mean age was 15.5 years old （ ranged from 10 to 18）. Microadenoma accounted for 80. 0% of the total patients, while macroadenoma accounted for 20. 0%. A total of 8.0% of adenomas in our study was invasive. Transsphenoidal approach surgery was commonly used for all the eases in our study, 2 of which used intra-operative navigation assistance. Total resection was achieved in 96. 0% of patients, and the rest achieved the subtotal resection. Forty-four （80. 0%） patients got full-term follow-up until 1 year after surgery. Conclusion Transsphenoidal approach surgery is the most commonly used and first line surgical therapy. The recurrence rate is about 15. 0%, and the patients need regular examination for pituitary hormones and imaging examination study to confirm the recurrence.