目的 探讨原发性肝血管肉瘤（primary hepatic angiosarcoma, PHA）的病理学特点及其鉴别诊断。 方法 对11例PHA患者的病理标本进行组织学观察和免疫组织化学染色。 结果 肉眼见肿瘤弥漫分布，或呈多结节/单结节，切面囊实性，见出血。镜下见肿瘤形成海绵状血管腔样结构，部分区域呈实性片状。瘤细胞大小不一，梭形或上皮样，核异型性明显，并形成多核巨细胞，实性区核分裂相易见（>5个/10HPF）。免疫组化：Vimentin、CD31和CD34均阳性，Desmine、CD117、AFP和CK均阴性，部分病例F-VIII相关抗原阳性。 结论 PHA是少见的血管内皮细胞来源的肝脏恶性肿瘤，组织学特征及免疫组织化学染色有助于明确诊断。
Abstract
Objective To investigate the clinicopathological features and differential diagnosis of primary hepatic angiosarcoma (PHA). Methods Eleven cases of PHA were investigated at the light microscopic level and were studied by immunohistochemical techniques. Results Macroscopically, tumors showed diffuse, multi-nodular or single nodular patterns. Heamorrhage were observed. Microscopically, the tumor composed of endothelial cells which formed the irregular vessels or cavernous vascular space. Tumor cells were spindle-shaped or epithelioid with atypical nuclei. Multinucleated cells and prominent mitosis were seen in some areas. Immunohistochemical staining showed expression of Vimentin，CD31 and CD34 in all cases. Some cases had F-VⅢ associated antigen expression. None of the cases had Desmin, CD117, AFP or CK expression. Conclusions PHA is a rare malignant heamangioendothelial tumor. Its histological and immohistochemical features are necessary for the confirmation of diagnosis.
关键词
肝脏 /
血管肉瘤 /
病理学
Key words
liver /
angiosarcoma /
pathology
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