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Abstract：
In 2013, the World Health Organization defined perivascular epithelioid cell tumor (PEComa) as \"a mesenchymal tumor which shows a local association with vessel walls and usually expresses melanocyte and smooth muscle markers.\" This generic definition seems to better fit the PEComa family, which includes angiomyolipoma, clear cell sugar tumor of the lung, lymphangioleiomyomatosis, and a group of histologically and immunophenotypically similar tumors that include primary extrapulmonary sugar tumor and clear cell myomelanocytic tumor. Clear cell tumors with this immunophenotypic pattern have also had their malignant variants described. When localizing to the liver, preoperative radiological diagnosis has proven to be very difficult, and most patients have been diagnosed with hepatocellular carcinoma, focal nodular hyperplasia, hemangioma, or hepatic adenoma based on imaging findings. Examples of a malignant variant of the liver have been described. Finally, reports of malignant variants of these lesions have increased in recent years. Therefore, we support the use of the Folpe criteria, which in 2005 established the criteria for categorizing a PEComa as benign, malignant, or of uncertain malignant potential. Although they are not considered ideal, they currently seem to be the best approach and could be used for the categorization of liver tumors.
摘要：
2013年，世界卫生组织将血管周围上皮样细胞瘤（PEComa）定义为“间充质肿瘤，与血管壁局部结合，通常表达黑素细胞和平滑肌标志物。“这个通用定义似乎更适合PEComa家族，包括血管平滑肌脂肪瘤，肺透明细胞糖肿瘤，淋巴管平滑肌瘤病,和一组组织学和免疫表型相似的肿瘤，包括原发性肺外糖肿瘤和透明细胞肌细胞瘤。具有这种免疫表型模式的透明细胞肿瘤也已描述了其恶性变体。当定位到肝脏时，术前放射诊断已被证明是非常困难的，大多数患者被诊断为肝细胞癌，局灶性结节增生,血管瘤,或基于影像学表现的肝腺瘤。已经描述了肝脏的恶性变体的实例。最后,近年来，这些病变的恶性变异的报道有所增加。因此,我们支持使用Folpe标准，2005年确立了将PEComa分类为良性的标准，恶性,或不确定的恶性潜力。虽然它们不被认为是理想的，它们目前似乎是最好的方法，可用于肝脏肿瘤的分类。
