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目的探讨原发性肾上腺血管肉瘤伴肾上腺皮脂腺瘤的临床与病理学特点。方法对1例原发肾上腺血管肉瘤伴肾上腺皮质腺瘤进行回顾,分析其临床表现、内分泌改变、影像学及病理组织学和免疫组化特点,并文献复习。结果患者因高血压3年余,影像学示右肾上腺肿物就诊。内分泌检查发现醛固酮/肾素比值升高。镜下表现为两种类型的肿瘤细胞,一种是以透明细胞及致密细胞混合而成,特征与良性肾上腺腺瘤一致;另一种则以大片弥漫出血为背景,浸润在肾上腺皮脂腺瘤细胞巢之间,形成大小及形状不规则血管腔,腔内可见红细胞,血管内衬单层非典型内皮细胞,可见核仁,与血管肉瘤一致。免疫组化示瘤细胞AE1/AE3、CD31(+),Ki-67阳性指数20%。结论原发性肾上腺血管肉瘤伴肾上腺皮脂腺瘤属于罕见病例,预后较差,CD31、CD34、FVIII和D2-40阳性可以帮助确诊。
Objective To investigate the clinical and pathological features of primary adrenal angiosarcoma with adrenocortical adenoma. Methods One case of primary adrenal angiosarcoma with adrenocortical adenoma was retrospectively analyzed, and its clinical manifestations, endocrine changes, imaging and histopathology and immunohistochemistry were reviewed and reviewed. Results Patients due to hypertension more than 3 years, imaging studies showed right adrenal glands. Endocrine examination found aldosterone / renin ratio increased. Microscopic appearance of two types of tumor cells, one is a mixture of transparent cells and dense cells, characterized by benign adrenal adenomas; the other is a large diffuse hemorrhage as the background, infiltration in the adrenal sebaceous cell nests Between the formation of irregular size and shape of the vessel cavity, cavity visible red blood cells, blood vessels lining atypical endothelial cells monolayer, visible nucleoli, consistent with angiosarcoma. Immunohistochemistry showed tumor cells AE1 / AE3, CD31 (+), Ki-67 positive index of 20%. Conclusions Primary adrenal angiosarcoma with adrenal sebaceous adenoma is a rare case with a poor prognosis. The positive results of CD31, CD34, FVIII and D2-40 can help confirm the diagnosis.