雄激素不敏感综合征手术治疗及探查结果分析

Analysis of surgical management and clinical pathological findings of androgen insensitivity syndrome

目的探讨雄激素不敏感综合征(androgen insensitivity syndrome,AIS)患者的性腺切除途径及其临床病理特点。方法回顾性分析1984年6月至2009年3月北京协和医院诊治的74例AIS患者的性腺切除途径、术后病理结果及性腺位置等特点。结果 74例患者均手术切除双侧性腺。其中完全型雄激素不敏感综合征(CAIS)48例,平均年龄22.0岁,有家族史者3例(6.25%),术中探查发现25例双侧性腺均位于盆腔(52.1%),13例双侧性腺均位于腹股沟内(27.1%);不完全型雄激素不敏感综合征(IAIS)26例,平均年龄16.5岁,有家族史者5例(19.2%),术中探查发现3例双侧性腺均位于盆腔内(11.5%),9例双侧性腺均位于腹股沟内(34.6%),10例双侧性腺均为于大阴唇中(38.5%)。CAIS患者中,开腹切除性腺21例,腹腔镜下性腺切除27例;IAIS患者中,9例开腹切除性腺,7例腹腔镜下性腺切除,10例经会阴切除性腺。术后病理证实睾丸肿瘤的发生率为18.9%,恶性肿瘤占4.1%。结论 IAIS的患者就诊和手术的平均年龄均较CAIS患者年轻,IAIS的患者较CAIS患者有家族史的多。双侧性腺位于盆腔的患者中CAIS多于IAIS,性腺降至腹股沟和大阴唇内的患者中IAIS多于CAIS。AIS患者性腺肿瘤发生率较高,一旦确诊应尽早手术治疗;可参考性腺的位置采用经会阴及腹腔镜微创手术切除性腺。

Objective： To investigate the route（s） of surgical gonadectomy and the clinical pathological character istics of androgen insensitivity syndrome （AIS）. Methods： Seventy-four patients with AIS treated in Peking Union Medical College Hospital from June 1984 to March 2009 were retrospectively studied, with focus on the analysis of route of gonadectomy, pathological features and locations of gonads. Results： All of 74 AIS patients had surgical resection of bilateral gonads. There were 48 cases of complete AIS （CAIS） and 26 cases of incomplete AIS （IAIS）. Among the patients with CAIS, the mean age was 22.0 years and 3 cases （6.25%） had a family history. There were 25 cases with bilateral gonads loca- ted in the pelvis （52.1%） and 13 cases in inguinal canal （27.1%）. In IAIS patients, the mean age was 16.5 years and 5 patients （19.2%） had a family cated in the pelvis （11.5%）, 9 in inguinal canal history. It was found that 3 cases with bilateral gonads lo （34.6%） and 10 in the labia major （38.5~）. 21 CAIS pa tients had undergone gonadectomy through abdominal operations and 27 by laparoscopy. However, among 26 IAIS patients, 9 patientrs gonads were removed through abdominal operations, 7 through laparoscopy and 10 through perineal operations. The incidence of testicular tumors was 18.9% and the malignant rate was 4.1% in all pathology specimens. Conclusions： The mean age for visiting a doctor and surgery in IAIS patients was younger than that in CAIS patients and there were more IAIS patients with a family history. The pelvic gonads were more common in CAIS patients, while more gonads tended to descend into inguinal canal or labia major in IAIS patients. The gonads should be removed right after diagnosed and the better choice of gonadectomy was through laparoscopy or perineal operations.