Clinical and ultrasound features of uterine perivascular epithelioid cell tumors: case series and literature review.

IF 6.1 1区 医学 Q1 ACOUSTICS
Ultrasound in Obstetrics & Gynecology Pub Date : 2024-11-01 Epub Date: 2024-10-12 DOI:10.1002/uog.29116
R Wang, H Luo, W Cao
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引用次数: 0

Abstract

Objective: To describe the clinical and ultrasonographic features of uterine perivascular epithelioid cell tumor (PEComa) using standardized terminology.

Methods: 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).

Results: 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 shape (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.

Conclusions: 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.

子宫血管周围上皮样细胞瘤的临床和超声特征:病例系列和文献综述。
目的: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: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2010年1月至2023年9月期间在中国四川大学华西第二医院经病理和免疫组化确诊的子宫PEComa患者的回顾性分析。采用形态学子宫声像图评估(MUSA)共识和国际子宫内膜肿瘤分析(IETA)共识对子宫PEComa的声像图特征进行标准化描述。我们总结了本中心病例以及2013年1月1日至2023年9月30日(含)期间使用PubMed进行的文献综述中发现的子宫PEComa的临床和超声特征:结果:我们的队列中有五名患者,年龄在 33-57 岁(中位数为 52 岁)之间,共有六个子宫 PEComa 病灶。所有病例均有完整的超声和病理图像。所有患者均无结节性硬化综合征病史。其中两名患者为恶性 PEComa(一名患者有两个病灶),三名患者为良性 PEComa,病灶来自宫颈、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、盆腔或腹部疼痛。三例良性 PEComa 患者分别接受了全子宫切除术和双侧附件切除术、肿瘤切除术和保守治疗,而两例恶性 PEComa 患者均接受了全子宫切除术和双侧附件切除术,随后接受了化疗。定期随访(6 至 24 个月)发现一例复发。两例病变被误诊为子宫肌瘤,两例被误诊为宫颈癌,一例被误诊为转移性宫颈癌(伴有子宫肌层浸润),还有一例诊断不明确。超声检查显示,大多数病变呈规则的圆形或卵圆形(66.7%),回声均匀(66.7%),低回声(66.7%),其中一个(16.7%)恶性宫颈息肉瘤呈囊性区域,一个(16.7%)良性宫颈息肉瘤呈点状钙化。所有病变均无阴影,大多数病变显示中等至丰富的血管(颜色评分为 3-4,占 83.3%)。在 100%的病例中,病变周边的颜色评分为 2-4,而在 83.3%的病例中,病变内部的颜色评分为 2-4。这三种良性 PEC 瘤的血管分布特征相似,内部血管散在分布,周边血管呈环状分布。文献检索发现了11篇文章,描述了18例子宫PEC瘤的超声波外观,其特征与我们队列中的病例相似:结论:子宫内膜异位瘤的声像图特征包括均匀或不均匀的低水肿性肿块,一般为圆形或卵圆形,边缘规则,偶见囊性区域或钙化,缺乏阴影,通常显示中等至丰富的血管。虽然子宫长纤维瘤的术前超声诊断仍具有挑战性,特别是考虑到此处描述的声像图特征的非特异性,但分散的瘤内血管和外周环形血管分布可作为子宫长纤维瘤的诊断线索,但需要更多病例来证实。© 2024 国际妇产科超声学会。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
12.30
自引率
14.10%
发文量
891
审稿时长
1 months
期刊介绍: Ultrasound in Obstetrics & Gynecology (UOG) is the official journal of the International Society of Ultrasound in Obstetrics and Gynecology (ISUOG) and is considered the foremost international peer-reviewed journal in the field. It publishes cutting-edge research that is highly relevant to clinical practice, which includes guidelines, expert commentaries, consensus statements, original articles, and systematic reviews. UOG is widely recognized and included in prominent abstract and indexing databases such as Index Medicus and Current Contents.
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