骨血管内皮恶性肿瘤

目的报道临床较为少见的骨血管内皮恶性肿瘤并讨论疾病的性质、诊断标准、治疗与预后。方法回顾我院1996-2001年5年间曾收治的4例骨血管内皮恶性肿瘤,报告其临床,影像学、病理学特点、治疗及随访结果,并作相应讨论。4例骨血管内皮恶性肿瘤患者均为男性,年龄14-59岁,平均38岁,发病部位分别位于股骨上段及股骨颈、颅骨顶部、拇指远节指骨、髂骨翼。其中骨上皮样血管肉瘤、骨血管肉瘤、骨上皮样血管内皮瘤、骨血管内皮瘤各1例。结果4例骨血管内皮恶性肿瘤1例行肿瘤刮除术,3例行肿瘤广泛切除术,4例患者均未行放疗、化疗或放弃放化疗建议。1例术后2年死于肿瘤远处转移;1例术后3年复发,再次手术后已无瘤生存1年8个月;1例术后4年复发,再次手术后已无瘤生存1年4个月;1例已元瘤生存5年3个月。结论骨血管内皮肿瘤的影像学无特异性表现,易与其它骨的良恶性肿瘤相混淆。骨(上皮样)血管肉瘤具有比骨(上皮样)血管内皮瘤更为恶性的生物学行为,预后更差,两者鉴别诊断的关键是病理学依据,骨(上皮样)血管肉瘤必须于早期采取包括肿瘤广泛切除、辅助放疗及化疗在内的综合治疗。

Malignant tumor of vascular endothelium of bone

Objective To review the clinical experience of malignant tumor of vascular endothelium of bone; the property, diagnostic standard, treatment and prognosis of this group of rare bone tumors were discussed. Methods The clinical radio-graphic and histological features, as well as treatment and follow - up outcome of 4 patients with malignant vascular endothelial tumors of bone admitted in our hospital during 1996 - 2001 were reviewed. Of the 4 cases,all were male, with the age averaged 38 (ranged from 14 to 59) .The bone involved included the femoral neck and upper femoral shaft, parietal bone of the skull, distal phalangeal bone of the thumb and lateral part of ilium. They consisted one case each of epithelioid angiosarcoma, angiosarcoma, ep-ithelioid hemangioendothelioma and hemangioendothelioma of bone. Results of the 4 cases. 3 were treated with wide resection, 1 had curettage of the lesion. They either did not accept or gave up to have adjuvant treatment of radiotherapy or chemotherapy. One case died of distant metastasis 2 years after operation,1 survived for 1 year and 8 months after the second operation due to recurrence 3 years later, 1 survived for 1 year and 4 months after the second operation 4 years later and 1 remained continuously disease free for 5 years and 3 months. Conclusion There were no specific radiographic appearances of malignant hemangioendothelial tumors of bone, which is easily to be mistaken for other benign or malignant tumors of bone; epithelioid angiosarcoma of bone had more invasive biological behaviors than epithelioid hemangioendothelioma of bone; the key of differential diagnosis was histological examination. For the treatment of epithelioid angiosarcoma,it is necessary to carry out early radical excision as well as adjuvant radiotherapy and chemotherapy.