关键词: Adult Children Ewing sarcoma Prognostic factors Survival Treatment

来  源:   DOI:10.1016/j.jbo.2024.100619   PDF(Pubmed)

Abstract:
UNASSIGNED: The present study evaluates the clinical outcomes of children, adolescents and adults with Ewing sarcoma and identifies the prognostic factors.
UNASSIGNED: Included in the study were 222 pediatric and adult patients diagnosed with Ewing sarcoma (EwS) who were followed up between 1992 and 2019, and whose data were analyzed retrospectively.
UNASSIGNED: The median age of 131 male and 91 female patients included in the study was 13 (1-64). The median follow-up duration of the survivors was 79 months (range, 11-182 months). The 3-year EFS rate of the 222 patients was 34 % (Confidence Interval (CI) (0.158-0.242 %) and the OS rate was 54 % (CI, 0.289-0.590 %). For the non-metastatic patients, the 3-year EFS rate was 47 % and the OS was 68 %, while for the metastatic patients the 3-year EFS rate was 13 % and the OS was 30 %. Of the patient sample, 81 (36, 5 %) survived, of whom 72 were continuously free of disease while the disease persisted in nine, and three developed a secondary neoplasm (2 of whom subsequently died while one survived disease-free). Of the 129 patients who relapsed with metastases and/or local recurrence, eight survived and are disease-free, nine are alive with uncontrolled disease; five were lost to follow-up and 107 died.
UNASSIGNED: The findings of the present study suggest metastatic disease at presentation and positive margins after surgery to be of prognostic significance in EwS. Disruptions in aggressive local treatments may reduce the chances of cure in EwS.
摘要:
本研究评估了儿童的临床结局,青少年和成人尤因肉瘤,并确定预后因素。
该研究包括222名诊断为尤文肉瘤(EwS)的儿童和成人患者,他们在1992年至2019年之间进行了随访,并对其数据进行了回顾性分析。
纳入研究的131名男性和91名女性患者的中位年龄为13(1-64)。幸存者的中位随访时间为79个月(范围,11-182个月)。222例患者的3年EFS率为34%(置信区间(CI)(0.158-0.242%),OS率为54%(CI,0.289-0.590%)。对于非转移性患者,3年EFS率为47%,OS为68%,而对于转移性患者,3年EFS率为13%,OS为30%。病人样本中,81(36,5%)存活,其中72人连续没有疾病,而9人持续患病,3人发展为继发性肿瘤(其中2人随后死亡,1人无病存活)。在129例因转移和/或局部复发而复发的患者中,八人幸存下来,没有疾病,9人患有不受控制的疾病;5人失去随访,107人死亡。
本研究的结果表明,在EwS中,转移性疾病的表现和术后切缘阳性具有预后意义。积极的局部治疗中断可能会降低EwS治愈的机会。
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