摘要目的 探讨骨原发性中间型血管内皮肿瘤病理特征、鉴别诊断和骨血管肿瘤的分类.方法 收集5例发生于骨的中间型血管内皮肿瘤,对其临床、病理、影像学和免疫组织化学特征进行分析,免疫标记包括CD31、CD34、平滑肌肌动蛋白(SMA)、波形蛋白、CK、上皮细胞膜抗原(EMA)6种抗体.结果 5例中间型血管内皮肿瘤中3例为卡波西型血管内皮瘤(患者均为儿童),2例网状血管内皮瘤(患者均为中老年人).4例位于四肢长骨,1例在锁骨.影像学(包括X线平片、CT、MRI)表现为交界性或低度恶性骨质破坏,骨皮质均有不同程度缺损,4例伴病灶内或周边成骨,其中l例有放射状骨针.病理组织学形态均与软组织相应的中问型血管内皮肿瘤相似,但大多伴病灶内或周围反应性骨形成.免疫组织化学5例CD31、CD34、波形蛋白均不同程度阳性,3例SMA阳性,CK、EMA均阴性.结论 骨原发性中间型血管内皮肿瘤是客观存在的,冈此骨血管肿瘤的分类应该逐步与软组织血管肿瘤分类相一致,在良性血管瘤和恶性血管肉瘤之间,增设一类中间型血管内皮肿瘤.骨中间型血管内皮肿瘤的生物学行为是否与软组织相应肿瘤一致,应通过长期随访资料的积累才能定论.

abstractsObjective To study the radiologic and pathologic features of primary intermediate hemangioendothelioma of the bone.Methods Five cases of primary intermediate hemangioendothelioma of bone encountered in the past three years were enrolled into the study.The clinical,radiologic,pathologic and immunohistochemical features of the tumors were reviewed.Results The patients included 3 children with Kaposiform hemangioendothelioma and 2 elderly with retiform hemangioendothelioma.Four of the cases affected long bones and the remaining case affected the clavicle.One case showed multifocal involvement of the humerus. Radiologically,the tumors showed borderline to low-grade bony destruction,with various degrees of cortical defect.Intralesional or perilesional bone formation was demonstrated in 4 cases and radial spicules were seen in 1 case.The histopathologic features of primary intermediate hemangioendothelioma of bone were similar to those of soft tissue, except for the presence of reactive bone formation.Immunohistochemically,the tumor cells were positive for CD31 (5/5),CD34 (5/5),vimentin (5/5) and smooth muscle actin (3/5) but negative for cytokeratin and epithelial membrane antigen. Conclusions Primary intermediate hemangioendothelioma of bone is a distinct entity and similar histologic classification applies as in its soft tissue counterparts.Comparison of the biologic behavior requires long-term follow-up studies.