Department of Pathology, Changhai Hospital, Second Military Medical University, Shanghai 200433, China.
Diagn Pathol. 2012 Mar 9;7:23. doi: 10.1186/1746-1596-7-23.
Perivascular epithelioid cells (PECs) are constantly present in a group of tumors called PEComas, including angiomyolipoma (AML), clear-cell "sugar" tumor (CCST) of the lung and extrapulmonary sites, lymphangioleiomyomatosis, clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres and rare clear-cell tumors of other anatomic sites. PECs have distinctive morphologic, immunohistochemical, ultrastructural and genetic characteristics, including an epithelioid appearance with a clear to granular cytoplasm, a round to oval, centrally located nucleus, and an inconspicuous nucleolus. PECs also express melanocytic and myogenic markers like HMB45 and smooth muscle actin. PEComa is rare in human, and multifocal PEComas (PEComatosis) is extremely rare. Up to now there have been only less than 5 cases described in the literature. Here we report a 46-year old Chinese woman who had PEComatosis arising from the genital tract and pelvis. To our knowledge, this is the first case of PEComatosis ever reported in China.
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血管周上皮样细胞（PECs）经常存在于一组称为PEComas 的肿瘤中，包括血管平滑肌脂肪瘤（AML）、肺和肺外部位的透明细胞“糖”瘤（CCST）、淋巴管平滑肌瘤病、镰状韧带/蒂状韧带的透明细胞黑素细胞肿瘤和其他罕见的解剖部位的透明细胞肿瘤。PECs 具有独特的形态、免疫组织化学、超微结构和遗传特征，包括具有透明到颗粒状细胞质的上皮样外观、圆形到椭圆形、中央定位的核和不明显的核仁。PECs 还表达黑色素细胞和肌源性标志物，如 HMB45 和平滑肌肌动蛋白。PEComa 在人类中很少见，多灶性 PEComas（PEComatosis）极为罕见。到目前为止，文献中仅描述了不到 5 例。在这里，我们报告了一例来自生殖道和骨盆的 PEComatosis 的 46 岁中国女性。据我们所知，这是中国首例报道的 PEComatosis。
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