子宫血管周上皮细胞瘤（PEComa）是一种罕见的间叶性肿瘤，具有独特的组织学、免疫组织化学和遗传学特征，临床表现无特异性，影像学特征不典型，术前诊断困难，而且容易被临床误诊为子宫肌瘤、子宫肉瘤或其他子宫肿瘤。文献报道的子宫PEComa患者例数较少，迄今对其尚无统一诊疗规范、共识可供参考，目前手术是其首选治疗方法，辅助治疗疗效迄今尚存在争议，使用雷帕霉素靶蛋白（m TOR）抑制剂对其进行靶向治疗，似乎取得较好临床疗效。笔者拟就PEComa起源、定义、流行病学特征及病因，以及子宫PEComa特点、分类标准、临床表现、影像学诊断和鉴别诊断与子宫PEComa治疗的最新研究现状进行阐述，旨在为该病的后续研究提供参考。

Uterine perivascular epithelioid cell tumor （PEComa）is a rare mesenchymal neoplasm characterized by distinctive histological，immunohistochemical，and genetic features，along with non-specific clinical manifestations and atypical imaging characteristics.Preoperative diagnosis is arduous，and it is frequently misdiagnosed as uterine fibroids，uterine sarcoma，or other uterine tumors.To date，only a limited number of cases of uterine PEComa have been reported，and there is no standardized consensus on diagnosis and treatment of PEComa.Surgery remains the preferred treatment option，and the efficacy of adjuvant therapy is still controversial.Nevertheless，recently，targeted therapy with mechanistic target of rapamycin （m TOR）inhibitors appears to have achieved superior efficacy.The author intends to elaborate on the origin，definition，epidemiological characteristics and causes of PEComa，as well as the characteristics，classification criteria，clinical manifestations，imaging diagnosis and differential diagnosis of uterine PEComa，and the latest research status of uterine PEComa treatment，aiming to provide reference for subsequent research on this disease.