Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
Diagn Pathol. 2007 Dec 3;2:45. doi: 10.1186/1746-1596-2-45.
Perivascular epithelioid cell tumor (PEComa), other than angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangioleiomyomatosis (LAM), is a very rare mesenchymal tumor with an unpredictable natural history. The uterus is the most prevalent reported site of involvement of PEComa-not otherwise specified (PEComa-NOS). To the best of our knowledge, about 100 PEComa-NOS have been reported in the English Language medical literature, of which 38 were uterine PEComa-NOS. These reported cases of uterine PEComa-NOS have usually shown clinically benign behavior, but 13 tumors, three of them associated with tuberous sclerosis complex (TSC), exhibited local aggressive behavior and four of them showed distant metastases.
We report the case of a 59-year-old woman, who presented with renal and pulmonary lesions seven years after the initial diagnosis of uterine leiomyosarcoma. Left nephrectomy and right middle lobe wedge resection were performed. Histological and immunohistochemical analysis of the renal and pulmonary lesions, in addition to retrospective re-evaluation of the previous uterine tumor, led to the final diagnosis of malignant uterine PEComa with late renal and pulmonary metastases. All three lesions had the typical histological appearance of PEComa-NOS showing a biphasic growth pattern with continuous transition between spindle cells and epithelioid cells, often arranged around vascular spaces. Immunohistochemically, the tumor cells of both phenotypes in all three lesions stained for melanocytic (HMB-45 and Melan-A/MART-1) and myoid (desmin, smooth muscle actin, and muscle-specific actin/all muscle actin/HHF-35) markers.
The findings indicate that despite the small number of reported cases, PEComas-NOS should be considered tumors of uncertain malignant potential, and metastases to other organs might become evident even several years after the primary diagnosis.
血管周上皮样细胞瘤（PEComa），除了血管平滑肌脂肪瘤（AML）、透明细胞糖细胞瘤（CCST）和淋巴管平滑肌瘤病（LAM）外，是一种非常罕见的具有不可预测自然史的间叶性肿瘤。子宫是最常见的PEComa 未特指（PEComa-NOS）受累部位。据我们所知，在英语医学文献中已经报道了大约 100 例 PEComa-NOS，其中 38 例为子宫 PEComa-NOS。这些报道的子宫 PEComa-NOS 病例通常表现出良性的临床行为，但 13 例肿瘤，其中 3 例与结节性硬化症（TSC）相关，表现出局部侵袭性行为，4 例表现出远处转移。
我们报告了一例 59 岁女性的病例，她在最初诊断为子宫平滑肌肉瘤七年后出现肾和肺病变。进行了左肾切除术和右中叶楔形切除术。对肾和肺病变的组织学和免疫组织化学分析，以及对先前子宫肿瘤的回顾性重新评估，导致最终诊断为恶性子宫 PEComa 伴晚期肾和肺转移。所有三个病变均具有典型的 PEComa-NOS 组织学表现，呈双相生长模式，梭形细胞和上皮样细胞之间连续过渡，常围绕血管空间排列。免疫组织化学染色显示，所有三个病变中的两种表型肿瘤细胞均表达黑色素细胞（HMB-45 和 Melan-A/MART-1）和肌源性标志物（结蛋白、平滑肌肌动蛋白和肌肉特异性肌动蛋白/全肌肌动蛋白/HHF-35）。
这些发现表明，尽管报道的病例数量较少，但 PEComa-NOS 应被视为恶性潜能不确定的肿瘤，即使在原发诊断后数年，其他器官的转移也可能变得明显。
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