使用标准化术语描述子宫血管周围上皮样细胞瘤（PEComa）的临床和超声特征。这是对中国四川大学华西第二大学医院经病理学和免疫组化诊断并确诊的子宫PEComa患者的回顾性分析，2010年1月至2023年9月。子宫形态超声评估（MUSA）共识和国际子宫内膜肿瘤分析（IETA）共识用于子宫PEComa超声特征的标准化描述。我们总结了2013年1月1日至2023年9月30日（含）期间本中心病例以及使用PubMed进行的文献综述中发现的子宫PEComa的临床和超声特征。 5名患者，年龄33-57岁（中位年龄52岁） ) 年，我们的队列中总共包括 6 个子宫 PEComa 病变。所有病例均有完整的超声及病理图像。没有患者有结节性硬化症病史。两名患者患有恶性 PEComa（一名患者有两个病变），三名患者患有良性 PEComa，起源于子宫颈、子宫肌层或子宫腔。患者出现的症状包括阴道分泌物增多、阴道出血以及盆腔或腹部疼痛。 3例良性PEComa患者分别接受全子宫切除和双侧附件切除、肿瘤切除和保守治疗，而2例恶性病例均接受全子宫切除和双侧附件切除并化疗。定期随访（6至24个月）发现1例复发。 2 个病灶被误诊为子宫肌瘤，2 个误诊为宫颈癌，1 个误诊为转移性宫颈癌（伴肌层浸润），1 个误诊为不确定。超声检查显示大多数病灶呈规则圆形或卵圆形（66.7%）、回声均匀（66.7%）和低回声（66.7%），其中1例（16.7%）恶性PEComa显示囊性区域，1例（16.7%）良性PEComa显示囊性区域。点状钙化。所有病变均缺乏阴影，大多数显示中度至丰富的血管分布（颜色评分为 3-4，83.3%）。 100% 的病例外围颜色评分为 2-4，83.3% 的病例颜色评分为内部。三种良性PEComas在血管分布上表现出相似的特征，内部血管散在，周围血管呈圆形。文献检索找到了 11 篇文章，描述了 18 例子宫 PEComa 的超声表现，与我们队列的特征相似。子宫 PEComa 的超声特征包括均匀或不均匀的低回声肿块，通常为圆形或卵圆形，边缘规则，偶尔含有囊性区域或钙化，缺乏阴影，通常显示中等至丰富的血管分布。虽然子宫PEComa的术前超声诊断仍然具有挑战性，特别是考虑到本文描述的超声特征的非特异性，但弥散的瘤内血管和周围圆形血管分布可能作为子宫PEComa的诊断线索，但需要更多的病例来证实。 © 2024 国际妇产科超声学会。© 2024 国际妇产科超声学会。

To describe the clinical and ultrasonographic features of uterine perivascular epithelioid cell tumor (PEComa) using standardized terminology.This was a retrospective analysis of patients with uterine PEComa diagnosed and confirmed by pathology and immunohistochemistry at West China Second University Hospital, Sichuan University, Sichuan, China, between January 2010 and September 2023. The Morphological Uterus Sonographic Assessment (MUSA) consensus and the International Endometrial Tumor Analysis (IETA) consensus were utilized for the standardized description of the sonographic characteristics of uterine PEComa. We summarized the clinical and ultrasound features of uterine PEComa in cases from our center and those found in a review of the literature conducted using PubMed from 1 January 2013 to 30 September 2023 (inclusive).Five patients, aged 33-57 (median, 52) years with a total of six uterine PEComa lesions were included in our cohort. All cases had complete ultrasonographic and pathological images. None of the patients had a history of tuberous sclerosis complex. Two patients had malignant PEComa (one patient had two lesions) and three had benign PEComa, originating from the cervix, myometrium or uterine cavity. Patients presented with symptoms including increased vaginal discharge, vaginal bleeding and pelvic or abdominal pain. The three patients with benign PEComa underwent total hysterectomy and bilateral adnexectomy, tumor excision and conservative management, respectively, while both malignant cases underwent total hysterectomy and bilateral adnexectomy followed by chemotherapy. Regular follow-up (from 6 to 24 months) revealed recurrence in one case. Two lesions were misdiagnosed as uterine fibroids, two as cervical cancer, one as metastatic cervical cancer (with myometrial invasion) and one was indeterminate. Ultrasound examination showed that most lesions displayed regular round or ovoid shapes (66.7%), uniform echoes (66.7%) and hypoechogenicity (66.7%), with one (16.7%) malignant PEComa showing cystic areas and one (16.7%) benign PEComa showing punctate calcifications. All lesions lacked shadowing and the majority showed moderate to abundant vascularity (color score of 3-4, 83.3%). The color score was 2-4 in the periphery in 100% of cases and internally in 83.3% of cases. The three benign PEComas showed similar characteristics in vascular distribution, with scattered internal vessels and peripheral vessels exhibiting a circular pattern. The literature search identified 11 articles describing the ultrasonographic appearance of 18 cases of uterine PEComa, with similar characteristics to those in our cohort.The sonographic features of uterine PEComa include a uniform or non-uniform hypoechogenic mass, typically round or ovoid with regular margins, occasionally containing cystic areas or calcifications, lacking shadowing and often showing moderate to abundant vascularity. Although the preoperative ultrasound diagnosis of uterine PEComa remains challenging, particularly given the non-specific nature of the sonographic characteristics described here, dispersed intratumoral vessels and a peripheral circular vascular distribution may serve as diagnostic clues for uterine PEComa, but more cases are needed for confirmation. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.© 2024 International Society of Ultrasound in Obstetrics and Gynecology.