原发性脾血管内皮肉瘤──附一例报告

The Splenic Primary Angiosarcoma──A Case Report

内脏血管内皮肉瘤是罕见病，占内脏肿瘤不到１％，原发于脾脏者国内仅见一例报告。临床主要表现为左上腹痛、脾肿大、贫血等非特异性症状。病灶有多发性和多脏器的特点，可复发及转移，但病程趋于良性，平均生存期４９个月，５年存活率为３０％，１０年存活率为５％。手术前常不能确诊。组织病理学一般可分为分化程度较高型和未分化型。肿瘤细胞释放相关Ⅷ因子是其生物学特征，所以相关Ⅷ因子抗原反应（ＦⅧ－ＲＡＧ）及荆豆凝集素有诊断及鉴别诊断价值。电镜及影像学检查可提高确诊率。主要治疗是手术切除，而单纯放疗和化疗效果较差。

Angiosarcoma is a rare neoplasm which originates in the splanchnic organizations,accounting for less than 1 per cent of the splanchnictumors. Only one case of the splenic angiosarcoma was reported in our country. Clinically,the main manifestation were pain of left upper abdomen,splenomegaly,anemia and so on,which were non-specific. Diagnosis of this tumor had usually not been confirmed until after operation. They may be classified into highly-differentiated type and undifferentiated type histopathologically. The positive results of FⅧ-RAG and UEA Ⅰ are of diagnostic value. Electron microscopy and radiography may enhance the accuracy rate of diagnosis. Angiosarcoma is a malignant tumor. Lesion may be multifocal,multiorganic;also be recurrant and metastatic. The clinical course had a benign presentation. the mean survival was reported to be 49 months with a 30 per cent survival at.5 year and 5 per cent at 10 years. The main method of treatment was resection by surgery,the simple chemotherapy or radiation therapy had poor results.