Acosta Andres Martin, Adley Brian P
From the Department of Anatomic/Clinical Pathology, University of Illinois at Chicago Hospital and Health Sciences System (Dr Acosta); and the Department of Pathology, Advocate Lutheran General Hospital, Park Ridge, Illinois (Dr Adley).
Arch Pathol Lab Med. 2017 Mar;141(3):463-469. doi: 10.5858/arpa.2016-0092-RS.
Perivascular epithelioid cell tumors (PEComas) are rare neoplasms that share phenotypic features with angiomyolipomas, clear cell sugar tumors, and lymphangioleiomyomatosis. They presumably represent the neoplastic counterpart of a yet-unidentified perivascular epithelioid cell that expresses smooth muscle and melanocytic immunomarkers. The uterus is the second most common site of origin for perivascular epithelioid cell tumors, after the retroperitoneum. Although most uterine perivascular epithelioid cell tumors are clinically benign and can be cured by a complete surgical excision, there is a subset characterized by both local and distant dissemination. Unfortunately, no single histopathologic or immunohistochemical parameter can accurately predict the clinical behavior of these tumors, which is why the 2012 World Health Organization classification of tumors of the female reproductive organs suggests the use of several criteria to predict the risk of aggressive clinical behavior. Here we review those perivascular epithelioid cell tumors of the uterine corpus with aggressive clinical behavior reported in the literature, and we discuss their most relevant clinical and histopathologic features.
血管周上皮样细胞瘤（PEComas）是一种罕见肿瘤，与血管平滑肌脂肪瘤、透明细胞糖瘤及淋巴管平滑肌瘤病具有共同的表型特征。它们可能代表一种尚未明确的血管周上皮样细胞的肿瘤对应物，该细胞表达平滑肌和黑素细胞免疫标志物。子宫是血管周上皮样细胞瘤的第二常见起源部位，仅次于腹膜后。尽管大多数子宫血管周上皮样细胞瘤临床呈良性，完整手术切除可治愈，但仍有一部分具有局部和远处播散的特征。遗憾的是，没有单一的组织病理学或免疫组化参数能够准确预测这些肿瘤的临床行为，这就是为什么2012年世界卫生组织女性生殖器官肿瘤分类建议使用多种标准来预测侵袭性临床行为风险的原因。在此，我们回顾文献中报道的具有侵袭性临床行为的子宫体血管周上皮样细胞瘤，并讨论其最相关的临床和组织病理学特征。
