摘要目的 探讨多发性内分泌腺瘤病1型(MEN-1)患者中肾上腺病变的发生率及临床特点.方法 回顾性分析1986年1月至2009年12月在北京协和医院内分泌科住院的32例MEN-1患者的腹部CT检查结果及临床特点.结果 50.0%MEN-1患者存在肾上腺形态异常.5例患者(31.3%)为双侧病变,其中1例为双侧腺瘤,2例为双侧增生,2例为一侧腺瘤一侧增生.11例患者(68.7%)为单侧病变,其中腺瘤占63.6%,腺瘤的平均直径为2.3 cm(0.8～4.0 cm),36.4%的患者表现为单侧增生、增粗.除1例肾上腺皮质腺瘤导致的库欣综合征、1例原发性醛固酮增多症腺瘤外,其余患者的肾上腺均为无功能病变.结论 中国人MEN-1患者中肾上腺病变的发生率与国外相似,但多为良性、无功能病变,鉴于国外报道中仍有一定比例的肾上腺皮质癌发生,因此建议MEN-1患者应常规进行肾上腺病变的筛查与随诊.

abstractsObjective To investigate the prevalence and characteristics of adrenal lesions in Chinese multiple endocrine neoplasia type 1 (MEN-1) patients. Methods Adrenal CT scan and clinical manifestations were retrospectively reviewed in 32 consecutive MEN-1 patients who were evaluated at our hospital during January 1986 to December 2009. Results Adrenal lesions were identified in 16 of 32 (50%) MEN-1 patients. Five (31.3%) patents with adrenal involvement showed bilateral lesions, including bilateral adenoma (n= 1), bilateral hyperplasia (n= 2) and adenoma and hyperplasia on each side (n= 2). Unilateral adrenal lesion was presented in 11 (68.7%) patients. Among which, 63.6% had adenomas with a mean diameter of 2.3 cm (0.8 - 4.0 cm) and the remainder was of hyperplasia or enlargement. In two patients, functioning adrenal abnormalities were detected including Cushing adenoma (n= 1) and aldosterone-secreting adenoma (n= 1). Conclusions The prevalence of adrenal lesion in MEN-1 patient is similar between China and western countries. These tumors are mostly benign, small and nonfunctioning. Taking into account a high incidence of adrenal carcinoma in previous foreign studies,rourine screening and close surveillance are still recommended for adrenal lesions in MEN-1 patients.