Fadare Oluwole
Department of Pathology, Wilford Hall Medical Center, Lackland Air Force Base, San Antonio, TX 78236, USA.
Int Semin Surg Oncol. 2008 Mar 6;5:7. doi: 10.1186/1477-7800-5-7.
In recent years, a group of tumors that have been designated "perivascular epithelioid cell tumors" (PEComa) have been reported with increasing frequency from a wide variety of anatomic locations. The uterus and retroperitoneum appear to be the most frequent sites of origin for these lesions. PEComas belong to an identically named family of tumors comprised of conventional angiomyolipomas, clear cell sugar tumors, lymphangiomyomatosis and clear cell myomelanocytic tumor of the falciform ligament/ligament teres, and are also known as PEComa-NOS. This article is a primer for clinicians on the most salient clinicopathologic features of uterine PEComas, as most of the debate and discussion have taken place in the pathologic literature. The author appraises in detail the current state of knowledge on PEComas of the uterus based on a review of published data on the 44 previously reported cases, and comments on areas of controversy. The latter are centered predominantly on the significant morphologic and immunophenotypic overlap that exists between uterine PEComa and some smooth muscle tumors of the uterus. The clinicopathologic features of cases reported as epithelioid smooth muscle tumors and cases reported as uterine PEComas are compared and contrasted, and a practical approach to their reporting is proposed.
近年来，一组被命名为“血管周上皮样细胞瘤”（PEComa）的肿瘤在各种解剖部位的报道频率日益增加。子宫和腹膜后似乎是这些病变最常见的起源部位。PEComas属于一个同名的肿瘤家族，该家族包括传统的血管平滑肌脂肪瘤、透明细胞糖瘤、淋巴管肌瘤病以及镰状韧带/圆韧带的透明细胞肌黑色素细胞瘤，也被称为PEComa-NOS。本文是为临床医生撰写的关于子宫PEComas最显著临床病理特征的入门读物，因为大多数争论和讨论都出现在病理文献中。作者在回顾44例先前报道病例的已发表数据的基础上，详细评估了子宫PEComas的当前知识状态，并对争议领域进行了评论。后者主要集中在子宫PEComa与一些子宫平滑肌肿瘤之间存在的显著形态学和免疫表型重叠上。比较并对比了报告为上皮样平滑肌瘤的病例和报告为子宫PEComas的病例的临床病理特征，并提出了一种实用的报告方法。
