摘要
目的总结原发性色素结节性肾上腺皮质病（primary pigmented nodular adrenocortical disease, PPNAD）的治疗经验。方法回顾性分析2005年1月至2017年1月收治的24例PPNAD患者的临床资料，男8例，女16例。年龄14～58岁，平均23岁。24例中23例具备库欣综合征典型临床表现，1例仅表现为高血压。8例患者临床可诊断为Carney综合征。内分泌相关实验室检查均符合促。肾上腺皮质激素非依赖性库欣综合征的特点。肾上腺CT检查提示双侧肾上腺多发结节11例，单侧。肾上腺多发结节4例，单侧肾上腺单发结节或肿物3例，肾上腺未见明显异常6例。结果24例患者均一期行腹腔镜下单侧肾上腺切除术或腹腔镜下单侧肾上腺肿物切除术。8例术后出现症状复发且血、尿皮质醇显著增高，其中5例行对侧肾上腺次全切除术，3例行对侧肾上腺全切除术。7例术后血、尿皮质醇轻度高于正常，但临床症状改善良好，遂未行二次手术，暂随访观察。9例术后恢复良好，未行二次手术，其中1例需长期激素替代治疗。结论双侧肾上腺全切除术加术后长期激素替代治疗为PPNAD的标准治疗方案，部分患者行单侧肾上腺切除术或肾上腺次全切除术亦可能得到长期缓解，但适宜人群仍需进一步探索。
Objective To discuss the surgical treatment of primary pigmented nodular adrenocortical disease（PPNAD）. Methods twenty-four cases of PPNAD were treated in our hospital from January 2005 to December 2017. Clinical data of these patients were reviewed. It included 8 males and 16 females with a mean age of 23 years old （ range 14 to 58 ）. 23 cases presented with typical symptoms of Cushing syndrome, 1 case presented with hypertension. Eight cases could be diagnosed with Carney complex. All cases were confirmed as ACTH-independent Cushing syndrome. Adrenal imaging showed bilateral multiple nodules in 11 cases, unilateral multiple nodules in 4 cases, unilateral single mass or nodule in 3 cases, normal adrenals in 6 cases. Results All of the 24 cases received laparoscope unilateral adrenalectomy or laparoseope unilateral mass resection. After the operation, 8 cases underwent secondary operation because of symptom recurrence and the elevated 24-hour urinary free cortisol. Among them, 5 cases received contralateral subtotal adrenaleetomy, 3 cases received contralateral total adrenaleetomy. Seven cases with a slightly elevated 24-hour urinary free cortisol but a good recovery of Cushing symptoms were followed-up. Nine cases recovered well after the first operation and they didn＇t undergo secondary surgical treatment, but 1 of the 9 cases needed glueoeorticoid replacement. Conclusions Bilateral adrenalectomy followed with long-term glucocorticoid replacement is the standard treatment of PPNAD. Unilateral adrenaleetomy or subtotal adrenaleetomy may he suitable for elected patients, but appropriate criteria need to be explored.
出处
《中华泌尿外科杂志》
CAS
CSCD
北大核心
2017年第4期264-267,共4页
Chinese Journal of Urology
