骨血管源性恶性肿瘤的临床分析

目的 回顾性分析临床上少见的血管源性恶性骨肿瘤的诊断、治疗和临床疗效.方法 从1998年10月至2008年11月我科诊治7例骨血管源性恶性肿瘤患者,包括5例血管内皮瘤和2例血管肉瘤.男4例,女3例;年龄19～76岁,平均42.6岁.1例为多中心病变,6例为单中心.1例病变位于椎体,其余6例位于四肢骨.所有患者均以局部疼痛就诊,病变影像学表现均为溶骨性破坏.其中2例血管肉瘤伴有软组织包块.1例脊柱病变接受边缘切除和术后放疗,5例接受广泛切除重建或截肢术,未行放化疗,另外1例放弃治疗.所有患者均经病理学诊断确诊,平均随访40个月(1～89个月).结果 股骨上段血管肉瘤患者半盆截肢后于术后17个月死于肺和内脏多发转移.脊柱血管内皮瘤患者术后3年死于其他原因.胫骨上端血管内皮瘤患者术后6年出现局部软组织复发,予以再次完整切除.保守治疗的患者带瘤生存,其他3例患者均无瘤生存.结论 骨的血管源性恶性肿瘤临床少见,主要根据病理学检查确诊,同一解剖部位的多中心病灶对诊断有一定特异性.手术广泛切除为治疗四肢病变的主要手段,椎体病变瘤内刮除后可辅以术后放疗.血管肉瘤恶性程度高、预后差.

Clinical analysis of malignant bone vascular tumors

Objective To retrospectively analyze the diagnosis, treatment and clinical outcomes of malignant tumors of blood vessels in bone. Methods From October 1998 to November 2008, 7 patients were diagnosed as malignant vascular tumors of bone, which include 4 hemangioendothehomas, and 2 an-giosarcomas. Their data was retrospectively reviewed. There were 4 males and 3 females, with the average age of 42.6 years, range from 19 to 65 years old. The lesions were located in the vertebral body (1 case) and extremities (6 cases). One patient was multiple lesions and the others were solitary. All patients suffered with local pain, and showed osteolytic lesions in X-ray, two of them associated with sofe tissue tumor. The patient, whose vertebral body was involved, underwent marginal resection and postoperative radiation. Among the ex-tremity patients, one patient with multiple lesions had not accepted any treatment, and the other 5 patients received wide resection or amputation. The diagnosis of all patients was confirmed by pathology. All patients were followed up 1-89 months (average 40 months). Results Among the patients, two died during the fol-low-up, one died due to lung or liver metastases at the 23rd month after the operation, and the other unrelat-ed to the disease three years after the operation. One recurred in the soft tissue six years after operation and got local resection. The untreated patient was alive with the tumor and lung metastases. The other patients remained disease free during follow-up. Conclusion The key of diagnosis is histological examination, there are some specific radiographic appearances help diagnose malignant vascular tumor of bone. It is essential to perform wide resection for malignant tumor of blood vessels, adjuvant radiotherapy could be considered for spinal patients with currottagc. The hemangioendothelioma seems to have better chnical outcome than an-giosarcoma.