骨恶性纤维组织细胞瘤是一种罕见的肿瘤,其特点是兼有组织细胞性和纤维性成分。男性发病率多于女性,无明显好发年龄,以中老年稍多。病损主要位于长骨骨端,约占全部病例的75%。病程较长,疼痛和逐渐长大的肿块是较早出现的临床症状。X线表现为溶骨性病变。诊断时要与骨纤维肉瘤鉴别。单纯手术治疗效果不满意,应辅以化疗或放疗。早期病例可在有效化疗控制下,行广泛病灶切除,保留肢体预后比骨肉瘤、骨纤维肉瘤好。 Bone malignant fibrous histiocytoma is a rare tumor characterized by both histiocytic and fibrous components. The incidence rate of males is higher than that of females, and there is no obvious age of occurrence. There are slightly more middle-aged and older people. The lesions are mainly located at the long bones, accounting for about 75% of all cases. The long course of the disease, the pain and the growth of the tumor is an early clinical symptoms. X-ray showed osteolytic lesions. When diagnosed with bone fibrosarcoma to identify. The effect of surgery alone is not satisfactory and should be supplemented by chemotherapy or radiotherapy. Early cases can be treated with effective chemotherapy and extensive resection of the lesions. The prognosis of retaining limbs is better than that of osteosarcoma and bone fibrosarcoma.