Yavuz Ekrem, Cakr Cağlar, Tuzlal Stk, Ahskal Bülent, Topuz Samet, Ilhan Rdvan
Department of Pathology, Istanbul Faculty of Medicine, Istanbul University, Topkapi, 34390 Istanbul, Turkey.
Appl Immunohistochem Mol Morphol. 2008 Jul;16(4):405-9. doi: 10.1097/PAI.0b013e318137a9c2.
We present a case of uterine perivascular epithelioid cell tumor (PEComa) coexisting with both pulmonary lymphangioleiomyomatosis and renal angiomyolipoma. The patient was a 42-year-old woman, suffering from massive abnormal uterine bleeding, progressive dyspnea, and arterial hypertension. Her clinical history included diagnoses of bilateral renal angiomyolipoma and pulmonary lymphangioleiomyomatosis. The patient underwent a subtotal hysterectomy and the specimen contained a subserosal tumor with irregular outlines in addition to uterine leiomyomata. The uterine tumor was histologically composed of solid sheets and tonguelike infiltration and lymphangioleiomyomalike structures within the myometrium, formed by epithelioid cells with ovoid to round nuclei and clear to light eosinophilic cytoplasms. The tumor did not display any morphologic sign of malignancy. The tumor cells showed expression of HMB-45 and smooth muscle markers. Ultrastructural analysis revealed abundant cytoplasmic microfilaments and rare small cytoplasmic bodies with a gridlike lining. We conclude that identification of lymphangioleiomyomalike growth pattern may aid in the differential diagnosis of uterine PEComa and suggest using both PEComa and smooth muscle tumor terminology.
我们报告一例子宫血管周上皮样细胞肿瘤（PEComa），同时合并肺淋巴管平滑肌瘤病和肾血管平滑肌脂肪瘤。患者为一名42岁女性，患有大量异常子宫出血、进行性呼吸困难和动脉高血压。她的临床病史包括双侧肾血管平滑肌脂肪瘤和肺淋巴管平滑肌瘤病的诊断。患者接受了子宫次全切除术，标本除子宫平滑肌瘤外，还包含一个浆膜下边界不规则的肿瘤。子宫肿瘤组织学上由实性片状和舌状浸润以及肌层内淋巴管平滑肌瘤样结构组成，由核呈卵圆形至圆形、胞质清亮至淡嗜酸性的上皮样细胞构成。肿瘤未显示任何恶性形态学迹象。肿瘤细胞显示HMB-45和平滑肌标志物表达。超微结构分析显示丰富的胞质微丝和罕见的带有网格状内衬的小胞质体。我们得出结论，识别淋巴管平滑肌瘤样生长模式可能有助于子宫PEComa的鉴别诊断，并建议同时使用PEComa和平滑肌肿瘤术语。
