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诊断时的转移性骨肉瘤:预后因素及长期结局——法国儿科经验

Metastatic osteosarcoma at diagnosis: prognostic factors and long-term outcome--the French pediatric experience.

作者信息

Mialou Valerie, Philip Thierry, Kalifa Chantal, Perol David, Gentet Jean-Claude, Marec-Berard Perrine, Pacquement Helene, Chastagner Pascal, Defaschelles Anne-Sophie, Hartmann Olivier

机构信息

Pediatrics Department, Leon Berard Center, Lyon, France.

出版信息

Cancer. 2005 Sep 1;104(5):1100-9. doi: 10.1002/cncr.21263.

Abstract

BACKGROUND

The objective of this report was to estimate long-term outcome and prognostic factors in children and adolescents who presented with metastatic osteosarcoma at diagnosis. Patients were treated in six French pediatric oncology centers with surgery and multiagent chemotherapy, mainly with high-dose methotrexate. Their medical records were reviewed retrospectively.

METHODS

The medical records of patients who were treated for metastatic osteosarcoma from 1987 to 2000 were reviewed. Patients were treated with the chemotherapy regimens recommended for nonmetastatic disease in children (the French Society of Pediatric Oncology OS 87 and OS 94 protocols) or, in a few patients, with other chemotherapy regimens. Surgical excision of the primary tumor and, when possible, of all metastatic sites was performed based on a personalized assessment of each patient's situation.

RESULTS

Seventy-eight patients age < 20 years were treated. Forty-six patients (59%) had only 1 metastatic site (35 to the lungs and 11 to bone). Twenty-eight patients (36%) achieved a complete remission after combination chemotherapy and surgery. The event-free survival and overall survival rates at 5 years were 14% and 19%, respectively. To date, 14 patients (18%) have remained alive with a median follow-up of 112 months. Pretreatment features associated with a shorter event-free survival in the multivariate analysis were metastasis to at least two organs and high alkaline phosphatase level. Patients with at least 1 of these poor prognostic factors had a 2.6% event-free survival rate at 5 years despite treatment.

CONCLUSIONS

The survival of patients with metastatic osteosarcoma were treated with conventional chemotherapy and surgery remained very poor. Patients should be classified into different prognostic groups and treated accordingly. New therapeutic approaches are warranted to improve the prognosis for patients with the most severe disease.

摘要

背景

本报告的目的是评估诊断时患有转移性骨肉瘤的儿童和青少年的长期预后及预后因素。患者在法国的六个儿科肿瘤中心接受手术和多药联合化疗,主要是大剂量甲氨蝶呤。对他们的病历进行了回顾性分析。

方法

回顾了1987年至2000年接受转移性骨肉瘤治疗的患者的病历。患者接受了推荐用于儿童非转移性疾病的化疗方案(法国儿科肿瘤学会OS 87和OS 94方案),或少数患者接受了其他化疗方案。根据对每位患者情况的个性化评估,对原发性肿瘤以及可能的所有转移部位进行手术切除。

结果

治疗了78名年龄小于20岁的患者。46名患者(59%)仅有1个转移部位(35个转移至肺部,11个转移至骨骼)。28名患者(36%)在联合化疗和手术后实现了完全缓解。5年时的无事件生存率和总生存率分别为14%和19%。迄今为止,14名患者(18%)仍然存活,中位随访时间为112个月。多变量分析中与无事件生存期较短相关的预处理特征是转移至至少两个器官和高碱性磷酸酶水平。尽管接受了治疗,但具有至少1项这些不良预后因素的患者5年时的无事件生存率为2.6%。

结论

接受传统化疗和手术治疗的转移性骨肉瘤患者的生存率仍然很低。应将患者分为不同的预后组并相应地进行治疗。需要新的治疗方法来改善最严重疾病患者的预后。

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