研究动态
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复发/进展性儿童尤文肉瘤的预后因素和结果:单中心 10 年经验。

Prognostic factors and outcome of relapsed/progressive pediatric Ewing sarcoma: single-center 10-year experience.

发表日期:2024 Aug 19
作者: Omar Arafah, Reem Ragab Hegazy, Moatasem El Ayadi, Azza Mohamed Nasr, Mohamed Fawzy
来源: Bone & Joint Journal

摘要:

尤文肉瘤(ES)是儿童和青少年中第二常见的原发性恶性骨肿瘤。尽管采用了更强化的化疗方案和改进的局部控制治疗,但仍然存在相当大的复发/进展率。一项回顾性研究,对 50 名在开罗大学国家癌症研究所 (NCI) 接受治疗的复发/进展性 ES 患者进行了回顾性研究。从2008年1月1日到2018年12月末,评估不同的预后变量和疾病结果。在50名符合条件的病例中,32名患者(64%)出现疾病复发,18名患者(36%)在治疗中出现疾病进展。中位随访期为 7.4 个月。中位总生存期 (OS) 为 7.5 个月,6 个月时的累积 OS 为 64%,1 年时的累积 OS 为 32.6%。 6 个月时的累积无事件生存率 (EFS) 为 41.3%,1 年时为 22.3%。疾病复发患者的 OS 和 EFS 优于疾病进展患者 (p = 0.019)。在复发/进展时接受局部控制的患者比仅接受化疗的患者具有显着更好的结果(p<<0.001)。自初次诊断起复发 > 2年是更好生存结果的唯一独立预测因素。复发/进展性ES患者预示着不良结果,治疗时疾病进展比复发更糟糕。在诊断后 2 年以上复发的患者、治疗中疾病复发而不是疾病进展的患者以及接受局部控制和强化化疗的患者中观察到更好的结果。© 2024。作者。
Ewing sarcoma (ES) is the second most common primary malignant bone tumor in children and adolescents. Despite more intensive chemotherapy regimens and improved local control therapy, there is still a considerable rate of recurrent/progressive disease.A retrospective study of 50 relapsed/progressive ES patients who were treated at the National Cancer Institute (NCI), Cairo University, during the period from 1st of January 2008 to the end of December 2018, to assess different prognostic variables and disease outcomes.Out of fifty eligible cases, 32 patients (64%) had disease recurrence, and 18 (36%) developed disease progression on treatment. The median follow-up period was 7.4 months. The median overall survival (OS) was 7.5 months, and the cumulative OS was 64% at 6 months and 32.6% at 1 year. The cumulative event-free survival (EFS) was 41.3% at 6 months and 22.3% at 1 year. Patients with disease recurrence had better OS and EFS than patients with disease progression (p = 0.019). Patients who underwent local control at relapse/progression had a significantly better outcome than patients who received chemotherapy only (p < 0.001). Recurrence > 2 years from initial diagnosis was the only independent predictor of better survival outcome.Patients with relapsing/progressive ES portended a poor outcome, with disease progression on treatment faring worse than relapse. Better outcome was observed in patients who experienced recurrence > 2 years after diagnosis, patients with disease recurrence rather than disease progression on treatment, and patients who underwent local control along with intensive chemotherapy.© 2024. The Author(s).