Department of Medical Oncology, İstanbul University Institute of Oncology, İstanbul, Turkey.
Department of Radiation Oncology, İstanbul University İstanbul Faculty of Medicine, İstanbul, Turkey.
Medicine (Baltimore). 2022 Dec 9;101(49):e32213. doi: 10.1097/MD.0000000000032213.
The prognosis of patients with Ewing's sarcoma family of tumors (ESFT) relapse is poor; the 5-year overall survival (OS) is 13%. We evaluated the effectivity of high-dose therapy (HDT) with autologous stem cell transplantation (ASCT) in adult patients with ESFT relapse. Between January 2010 and January 2021, we retrospectively analyzed 20 patients with ESFT who received HDT upon relapse. A combination of busulfan with melphalan was used as a conditioning regimen before ASCT. The median follow-up from diagnosis and from first relapse was 46.08 months (range; 10.71-186.87) and 14.41 months (range; 4.34-104.11), respectively. The median of age patients was 21.2 years (range, 17.6-25.3), and 10 (50%) patients were female. The tumor originated from the bone in 13 patients and soft tissue in 7 patients. Twelve patients had early (<2 years) relapse, and 8 patients had late (>2 years) relapse. Before HDT, 13 (65%) and 7 (35%) patients had pulmonary and extrapulmonary metastasis, respectively. After induction chemotherapy, 14 patients achieved complete response. The median OS1 and OS2 were 51.6 months (95% confidence interval [CI], range: 16.2-87) and 15.7 months (95% CI, range: 10.2-21.2), respectively. The 1-, 2-, and 5-year OS rates were 50%, 30%, and 15%, respectively. One patient died (sepsis) 1 month after ASCT. In univariate analyses, a disease-free interval (DFI) of < 2 years (P = .008) and incomplete response (P = .021) before ASCT were poor prognostic factors for OS2.HDT with ASCT can result in long-term survival of patients with ESFT relapse. HDT should be considered an important treatment opt ion in patients with a DFI > 2 years and complete response before transplantation.
尤文氏肉瘤家族肿瘤（ESFT）患者复发的预后较差，5 年总生存率（OS）为 13%。我们评估了大剂量化疗（HDT）联合自体造血干细胞移植（ASCT）在成人 ESFT 复发患者中的疗效。2010 年 1 月至 2021 年 1 月，我们回顾性分析了 20 例接受 HDT 治疗的 ESFT 复发患者。ASCT 前采用马法兰联合白消安作为预处理方案。从诊断到第一次复发的中位随访时间分别为 46.08 个月（范围：10.71-186.87）和 14.41 个月（范围：4.34-104.11）。患者的中位年龄为 21.2 岁（范围：17.6-25.3），10 例（50%）为女性。13 例（65%）肿瘤起源于骨，7 例（35%）起源于软组织。12 例（60%）为早期（<2 年）复发，8 例（40%）为晚期（>2 年）复发。HDT 前，13 例（65%）和 7 例（35%）患者分别有肺和肺外转移。诱导化疗后，14 例患者达到完全缓解。中位 OS1 和 OS2 分别为 51.6 个月（95%置信区间 [CI]，范围：16.2-87）和 15.7 个月（95%CI，范围：10.2-21.2）。1、2、5 年 OS 率分别为 50%、30%和 15%。1 例患者（败血症）在 ASCT 后 1 个月死亡。单因素分析显示，ASCT 前无疾病间隔时间（DFI）<2 年（P=0.008）和不完全缓解（P=0.021）是 OS2 的不良预后因素。HDT 联合 ASCT 可使 ESFT 复发患者获得长期生存。对于 DFI>2 年和移植前完全缓解的患者，HDT 应被视为重要的治疗选择。
