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原发性肾上腺平滑肌肉瘤:病例报告及文献复习

Primary adrenal leiomyosarcoma: case report and review of the literature.

作者信息

Wei Jianguo, Sun Aijing, Tao Jian, Wang Cheng, Liu Fang

机构信息

Shaoxing People's Hospital, Shaoxing, Zhejiang Province, People's Republic of China

Shaoxing People's Hospital, Shaoxing, Zhejiang Province, People's Republic of China.

出版信息

Int J Surg Pathol. 2014 Dec;22(8):722-6. doi: 10.1177/1066896914526777. Epub 2014 Mar 11.

DOI:10.1177/1066896914526777
PMID:24619012
Abstract

Primary adrenal leiomyosarcoma is a very rare tumor with an unusual location; the diagnosis is based entirely on histological and immunohistochemical evaluations. We report a primary adrenal leiomyosarcoma in a 57-year-old woman who was incidentally found to have an adrenal-occupying lesion during a routine medical examination. Computed tomography revealed a 64 mm × 77 mm soft tissue mass in the left adrenal gland. The patient underwent left radical nephroadrenalectomy. Histological examination of the tumor showed malignant spindle cells in interlacing fasicles and whorls. Nuclear pleomorphism, tumor giant cells, necrosis, and abnormal mitotic figures were present. On immunohistochemistry, the tumor cells were strongly positive for smooth muscle actin, desmin, and vimentin expression, but negative for cytokeratin, CD117, CD34, CD68, Myoglobin, S100, and HMB-45.The final histopathological diagnosis was primary adrenal leiomyosarcoma.

摘要

原发性肾上腺平滑肌肉瘤是一种非常罕见且位置特殊的肿瘤;诊断完全基于组织学和免疫组织化学评估。我们报告一例57岁女性的原发性肾上腺平滑肌肉瘤,该患者在常规体检时偶然发现肾上腺占位性病变。计算机断层扫描显示左肾上腺有一个64 mm×77 mm的软组织肿块。患者接受了左肾肾上腺根治性切除术。肿瘤的组织学检查显示在交错的束状和漩涡状结构中有恶性梭形细胞。存在核多形性、肿瘤巨细胞、坏死和异常有丝分裂象。免疫组织化学检查显示,肿瘤细胞平滑肌肌动蛋白、结蛋白和波形蛋白表达呈强阳性,但细胞角蛋白、CD117、CD34、CD68、肌红蛋白、S100和HMB-45呈阴性。最终组织病理学诊断为原发性肾上腺平滑肌肉瘤。

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