Department of Pathology, the Jikei University School of Medicine, Tokyo, Japan.
Arch Pathol Lab Med. 2009 Dec;133(12):1981-4. doi: 10.5858/133.12.1981.
A case of pigmented perivascular epithelioid cell tumor of the kidney in a 57-year-old woman with a clinically indicated tuberous sclerosis is presented. The lesion was a 3.0-cm, well-demarcated, black-colored mass. The tumor was histologically characterized by an epithelioid arrangement of round to polygonal cells with round nuclei and clear cytoplasm containing abundant melanin. Tumor cells showed mild atypia and low mitotic activity. A spindle cell proliferation was focally observed. There were no adipose elements or thick-walled vascular vessels. The stroma demonstrated intervening, thin, fibrovascular septa. Immunohistochemically, the tumor cells were strongly positive for HMB-45 but negative for epithelial and muscle markers, vimentin, and S100 protein. The patient had no evidence of disease 3 months after surgery. Pathologists and clinicians should know about the existence of pigmented perivascular epithelioid cell tumor of the kidney. This type of tumor should be differentiated from clear cell renal cell carcinoma or malignant melanoma.
本文报道了 1 例 57 岁女性临床提示结节性硬化症的肾脏色素性血管周上皮样细胞瘤。病变为 3.0cm 大小、边界清楚的黑色肿块。肿瘤组织学表现为圆形至多边形细胞的上皮样排列，细胞核圆形，细胞质透明，含有丰富的黑色素。肿瘤细胞轻度异型性，有低有丝分裂活性。局灶性观察到梭形细胞增殖。无脂肪成分或厚壁血管。间质显示分隔的、薄的纤维血管间隔。免疫组化染色显示，肿瘤细胞 HMB-45 强阳性，而上皮和肌肉标志物、波形蛋白和 S100 蛋白阴性。患者术后 3 个月无疾病证据。病理学家和临床医生应该了解肾脏色素性血管周上皮样细胞瘤的存在。这种肿瘤应与透明细胞肾细胞癌或恶性黑色素瘤相鉴别。
