Cossu A, Paliogiannis P, Tanda F, Dessole S, Palmieri G, Capobianco G
Eur J Gynaecol Oncol. 2014;35(3):309-12.
Perivascular epithelioid cell tumors (PEComas) are rare tumors characterized by co-expression of melanocytic and smooth muscle markers. PEComas have been reported in a wide variety of anatomic sites. In the female genital tract, PEComas most frequently affect the uterus. PEComas which occur in non-classic anatomic distributions are known as perivascular epithelioid cell tumor-not otherwise specified (PEComa-NOS). PEComas have an unpredictable biologic behavior, with some tumors being unresectable or metastatic at the time of diagnosis. The risk of aggressive behavior of these tumors has been linked to a number of factors evaluable on histopathological review following initial surgical resection. The authors report two cases of PEComa-NOS of the uterus: one with disease confined to uterus and the other case with lung and liver metastasis.
血管周上皮样细胞肿瘤（PEComas）是一种罕见肿瘤，其特征为黑素细胞和平滑肌标志物的共表达。PEComas已在多种解剖部位被报道。在女性生殖道中，PEComas最常累及子宫。发生于非典型解剖分布部位的PEComas被称为未另行指定的血管周上皮样细胞肿瘤（PEComa-NOS）。PEComas具有不可预测的生物学行为，一些肿瘤在诊断时即为不可切除或已发生转移。这些肿瘤侵袭性行为的风险与初次手术切除后组织病理学检查可评估的多种因素有关。作者报告了两例子宫PEComa-NOS病例：一例疾病局限于子宫，另一例伴有肺和肝转移。
