A Case Report of Malignant Perivascular Epithelioid Cell Tumors of the Uterus and Literature Review

IF 4.7 Q2 MATERIALS SCIENCE, BIOMATERIALS ACS Applied Bio Materials Pub Date : 2024-04-01 DOI:10.2147/IJWH.S453226
Daifeng Hu, Mengyue Miao, Hui Zhou, Xia Gu, Xuedan Wang, A. Teichmann, Qin Wang, Youzhe Yang
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Abstract

Abstract Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors originating from perivascular epithelioid cells. In gynecological system, the uterus is one of the most common sites affected by PEComas. Most PEComas are benign, and patients usually have a good prognosis. However, malignant uterus PEComa is rare, and better comprehensive epidemiological investigations are needed. To date, there are a few reported cases of uterus PEComa. We herein report a rare case of malignant PEComa occurred in the uterine corpus and cervix, possibly accompanied by pulmonary lymphangioleiomyomatosis (PLAM). In addition, 55 cases of malignant uterus PEComa were picked out and collected in the data base of PubMed and Medline. On the one hand, the age of onset, population distribution, clinical manifestations, metastatic sites and routes of metastasis were analysed. On the other hand, a summary of the epidemiology, pathogenesis, diagnosis, and treatments of uterus PEComa was given.
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子宫恶性血管周围上皮样细胞瘤病例报告及文献综述
摘要 血管周围上皮样细胞瘤(PEComas)是一种罕见的间叶肿瘤,起源于血管周围上皮样细胞。在妇科肿瘤中,子宫是最常见的受累部位之一。大多数 PEComas 是良性的,患者通常预后良好。然而,恶性子宫 PEC 瘤并不多见,因此需要进行更全面的流行病学调查。迄今为止,关于子宫长皮瘤的报道为数不多。我们在此报告一例罕见的发生在子宫体和子宫颈的恶性子宫长纤维瘤,可能伴有肺淋巴管瘤病(PLAM)。此外,我们还在 PubMed 和 Medline 数据库中挑选并收集了 55 例恶性子宫 PEComa 病例。一方面,分析了发病年龄、人群分布、临床表现、转移部位和转移途径。另一方面,对子宫息肉瘤的流行病学、发病机制、诊断和治疗方法进行了总结。
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来源期刊
ACS Applied Bio Materials
ACS Applied Bio Materials Chemistry-Chemistry (all)
CiteScore
9.40
自引率
2.10%
发文量
464
期刊介绍: ACS Applied Bio Materials is an interdisciplinary journal publishing original research covering all aspects of biomaterials and biointerfaces including and beyond the traditional biosensing, biomedical and therapeutic applications. The journal is devoted to reports of new and original experimental and theoretical research of an applied nature that integrates knowledge in the areas of materials, engineering, physics, bioscience, and chemistry into important bio applications. The journal is specifically interested in work that addresses the relationship between structure and function and assesses the stability and degradation of materials under relevant environmental and biological conditions.
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