原发性色素性结节状肾上腺皮质病一例报告并文献复习  

Case report of primary pigmented nodular adrenocortical disease and literature review

作　　者：李新德[1] 俞世成[1] 吴海洋[1] 张琦[1] 刘渊[1] 

机构地区：[1]浙江大学医学院附属邵逸夫医院泌尿外科,杭州310016

出　　处：《中华泌尿外科杂志》2008年第7期443-445,共3页Chinese Journal of Urology

摘　　要：目的 探讨原发性色素性结节状肾上腺皮质病（PPNAD）的临床表现及诊治方法。方法 PPNAD患者1例，男性，52岁。因血压升高1年余入院。库欣征面容。血、尿皮质醇升高，大、小剂量地塞米松抑制试验均不被抑制。B超检查示双侧肾上腺区未见肿块回声。CT检查左侧肾上腺可见1．6cm×2．0cm肿块，右侧正常。检索国内外医学数据库相关文献并复习。结果 患者行腹腔镜下左肾上腺切除术。术后病理标本切面中央见灰黑色细带，镜下示肾上腺皮质各带均增生、网状带细胞胞质内可见脂褐素颗粒。结合临床诊断为PPNAD。术后患者血压降至正常。目前仍在随访。结论 PPNAD临床较罕见，其症状体征可不典型，影像学检查多无特异性，临床上极易误诊。由于该病部分可合并多发性内分泌肿瘤综合征，故需对其加强认识。Objective To investigate the clinical features and the management of primary pig- mented nodular adrenocortical disease （PPNAD） and to evaluate its relationship with Carney complex. Methods One case of PPNAD reported. The patient was a 52 years old male. He was hospitalized because of hypertension for one year. The patient had a Cushing＇s face with elevated plasma and urine cortisone levels which could not be suppressed by both low dose and high dose dexamethasone tests. Ultrasonography showed normal bilateral adrenal glands. CT scan found a 1.6 cm× 2.0 cm mass in the left adrenal gland and normal contralateral adrenal gland. Results The patient had accepted left laparoscopic adrenalectomy. The pathological examination confirmed the diagnosis of PPNAD. Microscopic study showed that there were black-gray spots in the center of the specimen. Hyperplasia was found in all the three adrenal zones. Lipofuscin was observed in the cytoplasm of reticular zone cells. The patient＇s blood pressure had returned to normal level after the surgery. Conclusions PPNAD is a rare type of hypercortisolism. As there is no specific feature in clinical manifestation and radiological examination of this disease, it is very easy to make a misdiagnosis in clinical practice. PPNAD itself can be the comorbidity of Carney complex, careful differentiation is needed.

关 键 词：皮质醇增多症 原发性色素性结节状肾上腺皮质病 Carney综合征 

分 类 号：R586[医药卫生—内分泌] R734.2[医药卫生—内科学]