多中心骨巨细胞瘤影像学特点与治疗

Multicentric giant cell tumor：clinical analysis of 9 cases

目的 回顾性分析多中心骨巨细胞瘤的临床表现、影像学特点及预后.方法 1997至2010年在北京大学人民医院骨肿瘤科治疗的具有2个或2个以上独立病灶的骨巨细胞瘤9例,男5例,女4例,发病年龄14～37岁,平均23岁.3例患者年龄＜20岁.本研究共有29处肿瘤,每例患者平均有3.2处肿瘤（范围,2～10处）.最多者10处病变.4例（44%）仅有2处病变.2例患者为同时发生的病变.6例（67%）不同时发生的病变发生在2年以内.肿瘤部位依次是：脊柱、肋骨、股骨、胫骨、手足骨、腓骨、肱骨.有2例患者3处肿瘤位于骨干-干骺区或干骺区域.其中1例为骺板开放的未发育成熟的患者.所有多中心骨巨细胞瘤均有巨细胞瘤的组织学特点.根据肿瘤部位及Campanacci分级采用肿瘤刮除或整块切除的术式,并予以相应的内固定和重建方式.结果 9例患者29处肿瘤中有22处接受手术治疗,包括外院初次手术在内共进行31次手术.随访时间3～10年（平均6年）,2例共4处肿瘤在随访期内未发现复发,在总计29处肿瘤中有10处（34%）肿瘤复发,4例初次手术于外院进行.9例有7例至少有1处肿瘤复发,平均复发时间为2.5年（范围,2个月～9年）,6例在2年内出现复发.4例为1处肿瘤的单次复发,有3例出现多次复发.1例10处肿瘤患者死于放疗后恶性变,伴肺转移.结论 多中心骨巨细胞瘤发病年龄较单发骨巨细胞瘤更低,其每处病变的生物学行为都是独立的.肿瘤主要以沿血管轴向扩散、多中心起源的方式生长.对于多中心巨细胞瘤应坚持长期随访.

Objective To explore the clinical profiles, imaging features and prognosis of patient with multicentric giant cell tumor of bone （MGCT）. Methods Nine MGCT patients treated at our hospital between 1997 and 2010 were retrospectively examined. They had two or more separate lesions of giant cell tumor. There were 5 males and 4 females with an average age of 23 （ 14 -37 ） years. Three patients （33%） were under 20 years old. There were 29 tumors in the series with an average of 3.2 （ range, 2 - 10） per patient. Among 6 of them, a second lesion did not develop for 〉 2 years after initial presentation. There were 〉 2 sites of tumor involvement （n =4） and 10 lesions （n = 1 ）. Two patients had synchronous lesions involves 2 isolated locations. Twenty-nine lesions were located in spine, rib, femur, tibia, foot, hand, fibula and humerus. Three lesions of 2 patients involved metaphysic of long bones, one of whom was skeletally immature. All lesions had the typical histological features of giant cell tumor. The tumors were treated with curettage or resection was performed according to location and the Campanacci grade. Results Twenty-two lesions underwent surgery in all 29 lesions of 9 patients. A total of 31 operations were performed. The average follow-up period was 6 （3 - 10 ） years. Four lesions of 2 patients had no recurrence during follow-up. Ten lesions （34%） relapsed in all 29 lesions and 4 operations were performed firstly at other hospitals. Seven patients had one or more recurrences with an average recurrence time of 2. 5 years （ range, 2 months-9 years）. Six cases of local recurrence were within the first 2 years. Post-radiotherapy malignant transformation developed in 1 male patient with 10 lesions dying of malignant pulmonary metastasis. The total recurrence rate was similar to that of unifocal giant cell tumor. Conclusion MGCTs are likely to occur more in a younger population than unifocal giant cell tumors. And its risk of recurrence depends on the type of surgery. It is similar to the risk of recurrence for unifocal GCT. MGCT has vessel-axial distribution and multicentric generation. Long-term follow-up is necessary.