Malignant epithelioid angiomyolipoma ('Sarcoma ex angiomyolipoma') of the kidney - A case report and review of the literature
Malignant epithelioid angiomyolipoma ('Sarcoma ex angiomyolipoma') of the kidney - A case report and review of the literature
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DOI：
10.1097/00000478-200101000-00014
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发表时间：
2001-01-01
影响因子：
5.6
通讯作者：
Fletcher, CDM
中科院分区：
文献类型：
--
作者：
Cibas, ES;Goss, GA;Fletcher, CDM
摘要恶性上皮样血管平滑肌脂肪瘤是一种罕见的肾脏肿瘤。然而，它的存在受到质疑，基于不完整的恶性行为证据，缺乏相关的经典血管平滑肌脂肪瘤成分，或在某些情况下缺乏HMB-45的免疫反应性。我们描述了一个案件是HMB-45阳性，并出现在与一个经典的血管平滑肌脂肪瘤。患者接受肾部分切除术治疗。三年后，她出现了迅速增大的肝结节。肝脏细针穿刺证实了多形性上皮样细胞的存在，形态和化学特征与原发性肾肿瘤相同。在用阿霉素治疗两个周期后，肿瘤的大小减少了50%，表现状态显著改善。我们相信这个病例证实了恶性上皮样血管平滑肌脂肪瘤的存在。
Malignant epithelioid angiomyolipoma is a recently described rare tumor of the kidney. Its existence has been questioned, however, on the basis of incomplete evidence of malignant behavior, the absence of an associated classic angiomyolipoma component, or the absence of immunoreactivity for HMB-45 in some cases. We describe a case that was HMB-45-positive and arose in association with a classic angiomyolipoma. The patient was treated with a partial nephrectomy. Three years later, she developed rapidly enlarging liver nodules. A fine-needle aspiration of the liver confirmed the presence of pleomorphic epithelioid cells morphologically and immunohistochemically identical to those comprising the primary renal tumor. After two cycles of treatment with doxorubicin, there was a 50% reduction in the size of the tumors with marked improvement in performance status. We believe this case confirms the existence of a malignant epithelioid angiomyolipoma.