Koide O, Matsuzaka K, Tanaka Y
Clinical Laboratory, Division of Surgical Pathology, Tokyo Dental College Ichikawa General Hospital, Japan.
Pathol Int. 1998 Dec;48(12):998-1002. doi: 10.1111/j.1440-1827.1998.tb03874.x.
A recent case of angiomyolipoma (AML) with a prominent component of polygonal epithelioid cells is described. A 27-year-old Japanese male with tuberous sclerosis presented with massive abdominal tumors increasing progressively in size. The patient died of respiratory disturbance and the autopsy revealed massive tumors in the bilateral kidneys, liver and lymph nodes, subependymal giant cell glioma of the brain and lymphangiomyomatosis of the lungs. The giant tumors were an unusual type of AML with a component of polygonal epithelioid cells, which showed a hepatocellular carcinoma-like pattern in some areas. Smooth muscle components comprising spindle cells, short or plump spindle cells and polygonal epithelioid cells frequently exhibited positive staining for HMB-45 but negative staining for epithelial cell markers. The unusual AML presented in this case was thought to be of low-grade malignancy and slow growing. It has been suggested that angiomyolipomas with diffuse areas of epithelioid cell component are potentially malignant. Immunostainings positive for HMB-45 but negative for epithelial cell markers are considered to be useful in differentiating AML with polygonal epithelioid cell component from other tumors, especially from renal cell carcinoma and hepatocellular carcinoma.
本文描述了一例以多边形上皮样细胞为主要成分的血管平滑肌脂肪瘤（AML）。一名患有结节性硬化症的27岁日本男性，出现腹部巨大肿瘤，且肿瘤大小逐渐增大。患者死于呼吸功能障碍，尸检发现双侧肾脏、肝脏和淋巴结有巨大肿瘤，脑室内下巨细胞胶质瘤以及肺部淋巴管平滑肌瘤病。这些巨大肿瘤是一种不寻常类型的AML，含有多边形上皮样细胞成分，在某些区域呈现出肝细胞癌样的形态。由梭形细胞、短梭形或饱满梭形细胞以及多边形上皮样细胞组成的平滑肌成分，对HMB - 45常呈阳性染色，而上皮细胞标志物染色呈阴性。该病例中出现的这种不寻常的AML被认为是低级别恶性且生长缓慢。有人提出，具有弥漫性上皮样细胞成分区域的血管平滑肌脂肪瘤具有潜在恶性。HMB - 45阳性而上皮细胞标志物阴性的免疫染色，被认为有助于鉴别具有多边形上皮样细胞成分的AML与其他肿瘤，尤其是肾细胞癌和肝细胞癌。
