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

HE lei; ZHANG Wei; LIU Long-teng; CUI Di; LIU Dong-ge

doi:10.3969/j.issn.1007-8096.2017.08.009

Abstract：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-67 20％ +.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.