Objective To investigate the clinical characteristic, treatment and prognosis of undifferentiated high grade pleomorphic sarcoma of bone( BUPS) . Methods Seven cases of BUPS were analyzed retrospectively, including sites of tumor, image findings, pathology, surgical staging of tumor, treatment and results of follow-up, and the related lit-erature was reviewed. The preoperative traditional X-ray, CT, MRI, ECT and PET/CT were made. All cases were performed operation when BUPS was confirmed by puncturing biopsy or open biopsy. Results Tumors involved in distal femur in 2 cases, proximal femur in 3 cases, the proximal tibia and ilium each in 1 case. Radiologic findings of BUPS were nonspecific, mainly with a patchy or motheaten pattern of bone destruction. There were 2 cases in stage ofⅡA, 4 ⅡB and 1 Ⅲ. Hip disarticulation was performed in 3 cases, limb salvage was performed in 4 cases, and the chemotherapy was performed in all cases. The time of follow-up was 12~102 (45 ± 30) months, 3 cases developed distant metastasis and all of them with pathologic fracture, 2 cases died of lung metastasis two years after operation, the rest 4 cases were disease-free survival. Conclusions Surgical treatment is the primary method for BUPS and standard chemotherapy can improve survival rate. Pathologic fracture is associated with a poor prognosis.%目的：探讨骨未分化高级别多形性肉瘤( BUPS)的临床特征、治疗方法及预后。方法回顾分析7例BUPS患者的发病部位、影像学资料、病理、肿瘤的外科分期、治疗方法及随访结果等资料,并复习相关文献。患者术前行X线、CT、MRI、ECT或PET/CT检查,影像学表现缺乏特异性,主要以虫噬状或斑片状溶骨性骨破坏为主。穿刺活检或切开活检确诊为BUPS后均行手术治疗。结果病变累及股骨远端2例,股骨近端3例,胫骨近端及髂骨各1例,其中3例合并有病理性骨折。 Enneking分期：ⅡA期2例,ⅡB期4例,Ⅲ期1例。3例行髋关节离断术,4例行保肢术辅以化疗。7例均获随访,时间12~102(45±30)个月,7例患者中3例发生转移,均为病理性骨折的患者,其中2例术后2年死于肺转移,其余4例均无瘤生存。结论手术为BUPS的主要治疗手段,规范化疗有助于提高患者生存率。发生病理性骨折的患者预后欠佳。
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