Çakmakcı Selma, Sarı Neriman, Atasever Akkaş Ebru, Yıldız Fatih, Karakaya Ebru, Kaya Bektaş, Şafak Güngör Bedii, Berna Çakmak Öksüzoğlu Ömür, Ergürhan İlhan İnci
Department of Pediatric Hematology-Oncology, Dr. Abdurrahman Yurtaslan Training and Research Hospital, Ankara, Turkey.
Department of Radiation Oncology, Ministry of Health, Dr. Abdurrahman Yurtaslan Training and Research Hospital, Ankara, Turkey.
J Bone Oncol. 2024 Jul 6;47:100619. doi: 10.1016/j.jbo.2024.100619. eCollection 2024 Aug.
The present study evaluates the clinical outcomes of children, adolescents and adults with Ewing sarcoma and identifies the prognostic factors.
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.
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.
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.
本研究评估尤因肉瘤患儿、青少年和成人的临床结局，并确定预后因素。
本研究纳入了222例1992年至2019年间诊断为尤因肉瘤（EwS）的儿科和成人患者，对其数据进行回顾性分析。
本研究纳入的131例男性和91例女性患者的中位年龄为13岁（1 - 64岁）。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年无事件生存率（EFS）为34%（置信区间（CI）（0.158 - 0.242%），总生存率（OS）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%。在患者样本中，81例（36.5%）存活，其中72例持续无病，9例疾病持续存在，3例发生继发性肿瘤（其中2例随后死亡，1例无病存活）。在129例出现转移和/或局部复发的复发患者中，8例存活且无病，9例存活但疾病未得到控制；5例失访，107例死亡。
本研究结果表明，尤因肉瘤患者就诊时的转移性疾病和手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尤因肉瘤的治愈机会。
