Background: To evaluate patient and tumour characteristics, treatment, and their impact on survival in patients with multi-systemic metastases at initial diagnosis of high-grade osteosarcoma. Precedure: Eighty-three consecutive patients who presented with multi-systemic metastases at initial diagnosis of high-grade osteosarcoma were retrospectively reviewed. In cases of curative intent, the Cooperative Osteosarcoma Study Group recommended surgical removal of all detectable metastases in addition to complete resection of the primary tumour and chemotherapy. Results: Eighty-three eligible patients (1.8%) were identified among a total of 4605 individuals with high-grade osteosarcoma. Nine (10.8%) of these achieved complete surgical remission, of whom seven later had recurrences. The median follow-up time was 12 (range, 1–165) months for all patients. Actuarial event-free survival after 1, 2, and 5 years was 9.6 ± 3.2%, 1.4 ± 1.4%, and 1.4 ± 1.4%, and overall survival was 54.0 ± 5.6%, 23.2 ± 4.9%, and 8.7 ± 3.3%. In univariate analyses, elevated alkaline phosphatase before chemotherapy, pleural effusion, distant bones as metastatic sites, and more than one bone metastasis were negative prognostic factors. Among treatment-related factors, the microscopically complete resection of the primary tumour, a good response to first-line chemotherapy, the macroscopically complete resection of all affected tumour sites, and local treatment (surgery ± radiotherapy) of all bone metastases were associated with better outcomes. Tumour progression under first-line treatment significantly correlated with shorter survival times. Conclusion: The outlook for patients with multi-systemic primary metastases from osteosarcoma remains very poor. The utmost importance of surgical resection of all tumour sites was confirmed. For unresectable bone metastases, radiotherapy might be considered. In the patient group studied, standard chemotherapy was often insufficiently effective. In the case of such advanced disease, alternative treatment options are urgently required.
背景:本研究旨在评估初诊时已发生多系统转移的高级别骨肉瘤患者的临床特征、肿瘤特性、治疗方案及其对生存预后的影响。方法:回顾性分析83例初诊即伴有全身多系统转移的高级别骨肉瘤连续病例。对于有治愈可能的患者,骨肉瘤协作研究组建议在完整切除原发肿瘤及化疗基础上,手术切除所有可检测到的转移病灶。结果:在4605例高级别骨肉瘤患者中,共纳入83例符合条件者(占比1.8%)。其中9例(10.8%)实现完全手术缓解,但其中7例后续出现复发。全组患者中位随访时间为12个月(范围1-165个月)。1年、2年和5年精算无事件生存率分别为9.6±3.2%、1.4±1.4%和1.4±1.4%,总生存率分别为54.0±5.6%、23.2±4.9%和8.7±3.3%。单因素分析显示,化疗前碱性磷酸酶升高、胸腔积液、远处骨骼转移及多发性骨转移均为不良预后因素。在治疗相关因素中,原发肿瘤的显微镜下完全切除、对一线化疗的良好反应、所有病灶的肉眼完全切除以及所有骨转移灶的局部治疗(手术±放疗)与较好预后相关。一线治疗期间出现肿瘤进展与较短生存时间显著相关。结论:伴有初发多系统转移的骨肉瘤患者预后仍然极差。研究证实了手术切除所有肿瘤病灶的至关重要性。对于不可切除的骨转移灶,可考虑放疗干预。在本研究患者群体中,标准化疗方案常疗效不足。针对此类晚期病例,亟需探索替代治疗方案。