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目的研究肝恶性血管周上皮样细胞肿瘤的临床病理学特点、诊断与鉴别诊断。方法收集1例肝恶性血管周上皮样细胞肿瘤的临床病理资料,并复习相关文献,总结其临床病理特点及免疫组化情况。结果本例组织形态学表现为肿瘤间质富于毛细血管、血窦。肿瘤细胞以上皮样细胞为主,大部分细胞的胞质嗜伊红色,小部分细胞透亮,部分细胞质内可见褐色色素颗粒。细胞异型显著,核分裂象易见,并可见病理性核分裂及凝固性坏死。PAS染色(+)。免疫组化:肿瘤细胞HMB45和melanoma(pan)弥漫强(+),部分细胞vimentin和SMA(+),Ki 67增殖指数70%。结论肝恶性血管周上皮样细胞肿瘤是一种少见的肿瘤,诊断时应注意与其他肿瘤相鉴别,尤其是肝细胞癌、恶性黑色素瘤以及转移性肾透明细胞癌。
Objective To study the clinicopathological features, diagnosis and differential diagnosis of malignant perivascular epithelial cell tumor of liver. Methods One case of malignant perivascular epithelial cell carcinoma of the liver was collected. The clinical and pathological data of the patients were retrospectively reviewed. The clinicopathological characteristics and immunohistochemistry were summarized. The results of this case histological manifestations of the tumor stroma is rich in capillaries, sinusoids. Tumor cells to epithelial cells, most of the cytoplasm of eosinophilic cells, a small part of the translucent cells, some of the cytoplasm visible brown pigment particles. Cell abnormalities, mitotic figures easy to see, and visible pathological mitosis and coagulation necrosis. PAS staining (+). Immunohistochemistry: HMB45 tumor cells and melanoma (pan) diffuse strong (+), some cells vimentin and SMA (+), Ki 67 proliferation index 70%. Conclusions Hepatic malignant perivascular epithelial cell tumor is a rare tumor which should be distinguished from other tumors, especially hepatocellular carcinoma, malignant melanoma and metastatic renal clear cell carcinoma.