原发性色素性结节状肾上腺皮质病的临床病理特征:附5例报道

Primary pigmented nodular adrenocortical disease: a report of 5 cases

目的:回顾性研究5例原发性色素性结节状肾上腺皮质病(PPNAD)的临床病理特征。方法:分析临床和实验室资料,对病变组织行HE染色和免疫组化标记。结果:本组5例患者临床诊断为库欣综合征,其中4例合并Carney综合征;1例为特发性。病理学表现为多发性色素性皮质结节伴结节间皮质萎缩;镜检观察到两种细胞,一种为含有嗜酸性细胞质和脂褐素的大细胞,另一种为细胞质富含脂质,空泡状。这些细胞免疫组化标记神经元特异性烯醇化酶阳性(++)。结论:PPNAD可造成促肾上腺皮质激素(ACTH)非依赖性皮质醇增多症。且多数患有Carney综合征。

Objective To study the clinicopathologic characteristics of primary pigmented nodular adrenocortical disease （PPNAD）. Methods PPNAD in five cases were observed by light microscope and immunohistochemical staining. Results The 5 patients were diagnosed as Cushing＇s syndrome, 4 of whom were complicated with Carney＇s syndrome. The pathological characteristics were the presence of multiple pigmented nodules of cortical cells with atrophic cortical tissue. The nodules were composed of large granular eosinophilic cells containing hyperchromatin nuclei with prominent nucleoli and lipid-rich cells. The large cells were positive for neuron-specific enolase （NSE）, Conclusions PPNAD is a distinctive but rare form of adrenocorticotropic hormone （ACTH）-independent Cushing＇s syndrome. Most of such patients have Carney syndrome.