6例恶性骨巨细胞瘤的临床分析并复习文献

Clinical analysis of malignant giant cell tumor of bone in six cases and literature review

摘要:

目的：探讨恶性骨巨细胞瘤（malignant giant cell tumor of bone，MGCT）可能的发病机理、诊断和治疗方法。方法：选择2001年1月至2012年12月诊断明确并成功随访的MGCT患者6例，分析临床资料并回顾文献。结果：6例均为继发MGCT，首次发病到恶变间隔平均52.7个月。恶变结果：纤维肉瘤3例，骨肉瘤2例，恶性纤维组织细胞瘤1例。3例接受根治性手术，1例接受病灶边缘切除+辅助治疗，1例接受高强度聚焦超声治疗，1例接受化疗和姑息治疗。随访平均22.8个月。2例无疾病征象，2例带瘤存活，2例死亡。结论：MGCT约占骨巨细胞瘤（giant cell tumor of bone，GCT）总数的5%。放疗和反复不彻底的手术可能诱发GCT恶变。染色体失稳、异倍体出现和中心体畸变可能是恶变的重要因素。GCT患者在初次治疗后3年以上复发，需要怀疑恶变的可能。早期发现并行根治性手术，有望提高预后。

Abstract:

Objective：To explore the possible etiopathogenesis，diagnosis and treatment for malignant giant cell tumor of bone （MGCT）. Methods：Six cases from January 2001 to December 2012 were chosen，who had been clearly diagnosed as MGCT and were followed up successfully. Their clinical data were analyzed and relevant literatures were reviewed. Results：The six cases were all secondary MGCT. Average internal time from initial benign diagnosis to malignant transformation of giant cell tumor of bone was 52.7 months. Histopathologically，three cases presented with fibrosarcoma，two with osteosarcoma and one with malignant fibrous histiocy－toma. Treatments included radical surgeries for three，margin excision with adjunctive therapy for one，high intensity focus ultrasound therapy for one and chemotherapy with palliative treatment. Mean follow-up time was 22.8 months，two cases had no evidences of dis－eases，two were alive with tumors and two died. Conclusions：MGCT accounts for approximately 5％ of the total cases of giant cell tu－mor of bone（GCT）. Radiotherapy and recurrent incomplete curettage might induce malignant transformation. Chromosome instability，heteroploid appearance and centrosome aberration might play an important role in the malignant transformation. GCT displaying local recurrence over 3 years after the initial treatment should be considered the possibility of becoming malignant. Early detection and radi－cal surgery might improve the prognosis.