原发性色素性肾上腺结节增生四例诊治分析

Diagnosis and treatment of primary pigraented nodular adrenocortical disease report of 4 cases

目的探讨原发性色素性肾上腺结节增生（PPNAD）的诊断与治疗方法。方法回顾性分析2000--2007年收治的4例资料完整的PPNAD患者，包括临床表现、内分泌检查、手术方法及预后，结合文献复习，对此种疾病的诊断与治疗方法进行讨论。结果1例行双侧肾上腺全切除，术后需皮质激素替代，3例肾上腺次全切除者库欣症状缓解，不需服用激素，其中1例术后2年出现甲状腺癌。结论PPNAD是一类罕见的ACTH非依赖性库欣综合征，诊断依赖临床表现、内分泌检查及术后病理，症状明显者可采用肾上腺全切除，症状较轻者采用次全切除，术后要严密随访，注意有无其他内分泌器官病变。

Objective To study the diagnosis and treatment of primary pigmented nodular adrenocortical disease（PPNAD）. Methods The clinical data including symptom, endocrinal examination, surgical operation and prognosis of 4 cases of PPNAD hospitalized from 2000 to 2007 were analyzed respectively, relative literature were reviewed. Results 1 case received total adrenalectomy needed adrnacortical hormone postoperatively, 3 cases responded favorably to subtotal adrenalectomy and did not need adrnacortical hormone, however, 1 case was diagnosed of thyriod carcinoma 2 years after subtotal adrenalectomy. Conclusions PPNAD is a rare subtype of ACTH-independent Cushing, s syndrome, the diagnosis is depend on symptom, endocrinal examination and pathology. Total adrenalectomy is suitable to the patients with obvious symptom ; subtotal adrenalectomy to the patients with mild symptom, the lesion of other endocrinal organ must be followed up postoperatively.