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

Omar Arafah, Reem Ragab Hegazy, Moatasem El Ayadi, Azza Mohamed Nasr, Mohamed Fawzy
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Abstract

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

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复发/进展期小儿尤文肉瘤的预后因素和预后:单中心十年经验。
背景: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。尽管化疗方案更加密集,局部控制疗法也有所改善,但复发/进展性疾病的发生率仍然相当高:对2008年1月1日至2018年12月底期间在开罗大学国家癌症研究所(NCI)接受治疗的50例复发/进展期ES患者进行回顾性研究,以评估不同的预后变量和疾病结局:在符合条件的50例患者中,32例(64%)疾病复发,18例(36%)在治疗过程中出现疾病进展。中位随访期为 7.4 个月。中位总生存期(OS)为7.5个月,6个月和1年的累积OS分别为64%和32.6%。6个月和1年的累计无事件生存期(EFS)分别为41.3%和22.3%。疾病复发患者的OS和EFS均优于疾病进展患者(P = 0.019)。复发/进展期接受局部控制治疗的患者比仅接受化疗的患者的预后要好得多(p):复发/进展期 ES 患者的预后较差,治疗后疾病进展的情况比复发更差。诊断后 2 年以上复发的患者、治疗后疾病复发而非疾病进展的患者以及接受局部控制和强化化疗的患者的预后较好。
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来源期刊
CiteScore
3.50
自引率
0.00%
发文量
46
审稿时长
11 weeks
期刊介绍: As the official publication of the National Cancer Institute, Cairo University, the Journal of the Egyptian National Cancer Institute (JENCI) is an open access peer-reviewed journal that publishes on the latest innovations in oncology and thereby, providing academics and clinicians a leading research platform. JENCI welcomes submissions pertaining to all fields of basic, applied and clinical cancer research. Main topics of interest include: local and systemic anticancer therapy (with specific interest on applied cancer research from developing countries); experimental oncology; early cancer detection; randomized trials (including negatives ones); and key emerging fields of personalized medicine, such as molecular pathology, bioinformatics, and biotechnologies.
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