Tynski Zofia, Chiang Way, Barrett Albert
Department of Pathology, Clarion Hospital, Clarion, PA 16214, USA.
Department of Family Medicine, Clarion Hospital, Clarion, PA 16214, USA.
Case Rep Pathol. 2018 May 29;2018:5749421. doi: 10.1155/2018/5749421. eCollection 2018.
Malignant PEComas are rare mesenchymal neoplasms. These tumors harbor distinct myomelanocytic phenotype. The PEComa family of tumors includes lymphangioleiomyomatosis, angiomyolipoma, clear cell sugar tumor of the lung, and myomelanocytic tumor of the falciparum ligament/ligamentum teres. PEComas have no known normal cell counterpart. Majority of PEComas are benign and occur predominantly in the middle-age women. These tumors are commonly encountered in the uterus. Herein, we report a 20-year-old woman with a left inguinal mass metastatic to orbit, brain, lumbar spine, and skin at presentation. To our knowledge, this is the first case of metastatic PEComa to the orbit. This is the third case of primary PEComa of the inguinal area.
恶性PEComa是罕见的间叶性肿瘤。这些肿瘤具有独特的肌黑素细胞表型。PEComa肿瘤家族包括淋巴管平滑肌瘤病、血管平滑肌脂肪瘤、肺透明细胞瘤和镰状韧带/圆韧带的肌黑素细胞瘤。目前尚不知道PEComa有正常的细胞对应物。大多数PEComa是良性的，主要发生在中年女性。这些肿瘤常见于子宫。在此，我们报告一名20岁女性，就诊时左腹股沟肿块已转移至眼眶、脑、腰椎和皮肤。据我们所知，这是首例眼眶转移性PEComa。这是腹股沟区原发性PEComa的第三例病例。
