摘要
目的 :探讨原发性色素性结节状肾上腺皮质病 (PPNAD)的临床表现和诊断治疗方法。方法 :总结 4例PPNAD的临床资料 ,4例均有库欣综合征的临床表现 ,内分泌检查结果提示为功能自主性肾上腺皮质肿瘤 ,但影像学检查并未发现肾上腺肿瘤。结果 :4例患者均行单侧肾上腺全切除术 ,手术标本均表现为肾上腺大小正常或轻度增大 ,外表和切面上见黑色或深褐色小结节 ,结节间皮质萎缩 ,光镜下见组成结节的细胞体积大 ,胞质嗜伊红染色、颗粒状 ,部分细胞胞质中的颗粒状色素颗粒具脂褐质染色特征。结论 :PPNAD在青少年中是一种引起库欣综合征的罕见病因 ,双侧肾上腺切除术是治愈本病的方法。
Purpose:To probe into the pathogeny, pathology, symptoms, diagnosis and treatment of PPNAD. Methods:4 cases of PPNAD were reported and they (two males and two females, ages 12-38 years) had clinical features of the Cushings syndrome. Results of biochemical tests suggested the presence of an autonomously functioning adrenocortical neoplasm. However, radiologic examination of the adrenals did not show an adrenal tumor. Results:The 4 patients underwent unilateral total adrenalectomy. The adrenal pathologic findings in these patients were similar. Gross findings included: 1) normal or slightly increased total gland weight; 2) studding of the external and cut surfaces by small black, brown nodules; and 3) cortical atrophy. Microscopically, the nodules were composed predominantly of enlarged, globular, cortical cells with granular eosinophilic cytoplasm that often contained lipofuscin. Conclusions:PPNAD is a rare cause of Cushings syndrome in children and young adults, bilateral adrenalectomy is the treatment of choice.
出处
《临床泌尿外科杂志》
2003年第5期271-273,共3页
Journal of Clinical Urology
