Perivascular epithelioid cell neoplasm (PEComa) of the uterus: A systematic review

Perivascular epithelioid cell neoplasm (PEComa) of the uterus: A systematic review
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DOI:
10.1016/j.ijsu.2015.05.002
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发表时间:
2015-07-01
影响因子:
15.3
通讯作者:
D'Andrea, Vito
D'Andrea, Vito
中科院分区:
医学2区
文献类型:
--
作者:
Musella, Angela;De Felice, Francesca;D'Andrea, Vito

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背景:血管周围上皮样细胞肿瘤是一种罕见的间叶性肿瘤。妇科PEComa占文献报道的全部PEComa病例的四分之一以上。手术是最推荐的主要治疗方法,而辅助治疗通常用于高危病例。然而,这种肿瘤的最佳治疗方法尚未得到很好的确立,主要是因为迄今为止描述的病例很少。目的:本系统综述的目的是总结迄今为止关于PEComa的病因、临床和病理特征的已知信息,同时关注子宫病例最有效的治疗方案。数据来源:发表在各种期刊上的PEComas的文章在过去的70 years进行了analysed.Conclusions和关键发现:虽然妇科PEComas的最佳治疗是有争议的,手术切除仍然是基石。在高危患者中使用辅助治疗是必要的,以增加疾病控制。多学科的方法应该是关于妇科PEComa的治疗决策的关键。(C)2015年出版集团有限公司。由爱思唯尔有限公司出版。保留所有权利。
Background: Perivascular epithelioid cell neoplasm (PEComa) is a rare mesenchymal tumor. Gynecological PEComas account for just over one-fourth of the overall PEComa cases reported in the literature. Surgery is the most recommended primary treatment while adjuvant therapy is generally reserved for high-risk cases. However, the best management of this neoplasia has not been well established, primarily because of the paucity of cases described to date.Objectives: The aim of this systematic review is to summarize what is known thus far regarding the etiopathogenesis, clinical and pathologic features of PEComas, focusing also on the most valid treatment options for uterine cases.Data sources: Pubmed articles on PEComas published in various journals over the past 70 years were analyzed.Conclusions and key findings: Although the optimal treatment of gynecological PEComas is controversial, surgical resection remains the cornerstone. The use of adjuvant treatment is warranted in high risk patients to increase disease control. A multidisciplinary approach should be key in treatment decision-making regarding gynecological PEComas. (C) 2015 IJS Publishing Group Limited. Published by Elsevier Ltd. All rights reserved.