单中心107例骨原发血管源性肿瘤临床病理分析

Primary vascular tumors of bone: clinicopathologic analysis of 107 patients from single center institution

目的 探讨骨原发血管源性肿瘤的临床病理特征及诊断要点。方法 回顾性分析北京积水潭医院单中心107例骨原发血管源性肿瘤临床、影像学、组织学及免疫表型特点。结果 107例骨原发血管源性肿瘤中男性65例,女性42例,其中70例普通血管瘤,19例上皮样血管内皮细胞瘤,13例血管肉瘤,2例Kaposi型血管内皮细胞瘤,1例上皮样血管瘤,1例假肌源性血管内皮细胞瘤,1例Gorhams病。患者年龄3～80岁（平均39岁）,接近50%血管源性肿瘤位于中轴骨及骨盆,18例为多发。影像学表现以溶骨性改变为主,可以伴不同程度的周围硬化。普通血管瘤由于形态学特点可以直接诊断,部分非普通血管瘤病例需做免疫组化证实。92%病例CD31呈阳性（33/36）和83%病例CD34呈阳性（30/36）。结论 骨原发血管源性肿瘤在中国人群中发病率与西方国家相似,血管瘤、上皮样血管内皮细胞瘤和血管肉瘤是其主要亚型。结合影像学和病理形态学特点及免疫表型,有利于骨原发血管源性肿瘤诊断。

Purpose To investigate the clinicopathologic features, diagnosis of primary vascular tumors of bone. Methods The records, images, slides and immunohistochemistry stains of patients of primary vascular tumors of bone diagnosed were reviewed retrospectively. Results There were 65 males and 42 females ranging in age from 3 to 80 years （ mean, 39 years）. There were 70 cases of hemangioma, 19 epithclioid haemangioendotheliomas, 13 angiosarcomas, 1 epithelioid hemangioma, 2 kaposifornl hemangioendothelio- mas, 1 Gotham＇ s disease, and 1 pseudomyogenic haemangioendothelioma. Nearly fifty percent of the vascular tumors were localized in the axial bone and pelvis. 18 patients were multifocal lesions. Radiology showed the lytic features with variant sclerotic rims. Some cases could be directly diagnosed by histology. Immunohistochemistry staining CD31 was positive for 92% （33/36） and CD34 positive for 83% （30/36） except for common hemangioma patients. Conclusion The ratio of vascular tumors in all primary bone tumors trea- ted at institution is similar to that in western countries. Hemangioma, epithelioid haemangioendothelioma and angiosarcoma are the main types of vascular tumors of bone. The results of the present study suggest that radiology and histology provide favorable support and immunohistochemistry is very helpful for diagnosis.