腹腔恶性血管周上皮样细胞肿瘤合并转移性肝癌一例并文献学习

A Case of Malignant Perivascular Epithelioid Cell Tumor in the Abdominal Cavity Combined with Metastatic Liver Cancer and Literature Review

摘要: 血管周上皮样细胞肿瘤(PEComa)是起源于间质的罕见肿瘤，大多数良性，少数呈恶性。血管周上皮样细胞肿瘤多发于子宫、膀胱。血管周上皮样细胞肿瘤临床表现多无特异性，大多经CT等检查发现。本次分享的肾血管周上皮样细胞肿瘤为恶性，经开腹腹腔巨大肿瘤切除 + 左肾切除 + 肝左外叶切除 + 右肝部分切除 + 肝脏微波射频消融后病检确诊。

Abstract: Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal-origin tumor, most of which are benign, with a few presenting as malignant. PEComas most frequently occur in the uterus and bladder. The clinical manifestations of PEComa are mostly nonspecific and are often discovered through imaging such as CT scans. The renal PEComa shared in this case was malignant and was pathologically confirmed after open abdominal surgery involving resection of a large abdominal tumor, left lateral hepatectomy, partial resection of the right liver, and microwave radiofrequency ablation of the liver.