原发性肾上腺血管肉瘤伴肾上腺皮质腺瘤临床病理观察

Primary adrenal angiosarcoma arising in an adrenal cortical adenoma: a case report and review of the literature

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 study the clinicopathological features of primary adrenal angiosarcoma arising in an adrenal cortical adenoma. Methods A retrospective study was performed to analyze the clinical,radiological,histopathological features in a case of primary adrenal angiosarcoma arising in an adrenal cortical adenoma,with review of the literature.Immunohistochemistry was used to further confirm the diagnosis with follow-up. Results A 43-year-old man presented with hypertension for 3 years leading to the discovery of a mass-appearing right adrenal tumor. Endocrine investigations revealed aldosterone/renin ratio increased. On microscopic examination,2 tumor cell populations were identified. The majority of the lesion was composed of nests of monotonous polygonal cells resembling those of the zona fasciculata,traversed by a fine fibrovascular network; features in keeping with a benign adrenal cortical adenoma. Within the center of the lesion there was extensive hemorrhage and a second population of tumour cells,peripherally infiltrating between the nests of adrenal cortical adenoma cells and forming complex,anastomosing vascular channels. Red blood cells were observed within the vascular spaces. Atypical endothelial cells displayed moderate nuclear grade. On immunohistochemical analysis,the angiosarcomatous component expressed endothelial markers CD31 and epithelial marker AE1/AE3,Ki-6720% ＋. The features were consistent with an angiosarcoma,intermingling with the cords and nests of the adrenal cortical adenoma. Conclusion Primary adrenal angiosarcoma arising in an adrenal cortical adenoma is an extremely rare neoplasm with poor prognosis. Further immunohistochemical staining can basically confirmed the diagnosis.