Stachelek Gregory C, Ligon John A, Vogel Jennifer, Levin Adam S, Llosa Nicolas J, Ladle Brian H, Meyer Christian F, Terezakis Stephanie A, Morris Carol D, Ladra Matthew M, Pratilas Christine A
Department of Radiation Oncology and Molecular Radiation Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Department of Pediatric Oncology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Sarcoma. 2021 Apr 17;2021:6681741. doi: 10.1155/2021/6681741. eCollection 2021.
The majority of patients with localized Ewing sarcoma will remain disease-free long term, but for those who suffer recurrence, successful treatment remains a challenge. Identification of clinicopathologic factors predictive of recurrence could suggest areas for treatment optimization. We sought to describe our experience regarding predictors of recurrence and patterns of first failure in patients receiving modern systemic therapy for nonmetastatic Ewing sarcoma.
The medical records of pediatric and adult patients treated for localized Ewing sarcoma between 1999 and 2019 at Johns Hopkins Hospital were retrospectively analyzed. Local control was surgery, radiotherapy, or both. Recurrence-free survival (RFS) was calculated using the Kaplan-Meier method. Univariable and multivariable Cox proportional-hazards modeling was performed to obtain hazard ratios (HR) for recurrence.
In 94 patients with initially localized disease, there were 21 recurrences: 4 local, 14 distant, and 3 combined. 5-year and 10-year RFS were 75.6% and 70.5%, respectively. On multivariable analysis including age at diagnosis and tumor size, <95% tumor necrosis following neoadjuvant chemotherapy (NAC; HR 14.3, = 0.028) and radiological tumor size change during NAC (HR 1.04 per 1% decrease in size change, = 0.032) were independent predictors of recurrence. Among patients experiencing distant recurrence, pulmonary metastases were present in 82% and were the only identifiable site of disease in 53%.
Poor pathologic or radiologic response to NAC is predictive of recurrence in patients with localized Ewing sarcoma. Suboptimal tumor size reduction following chemotherapy provides a means to risk-stratify patients who do not undergo definitive resection. Isolated pulmonary recurrence was a common event.
大多数局限性尤因肉瘤患者长期无病生存，但对于复发患者，成功治疗仍是一项挑战。识别预测复发的临床病理因素有助于优化治疗方案。我们旨在描述接受现代全身治疗的非转移性尤因肉瘤患者复发的预测因素及首次失败模式。
回顾性分析1999年至2019年在约翰霍普金斯医院接受局限性尤因肉瘤治疗的儿童和成人患者的病历。局部控制采用手术、放疗或两者结合。采用Kaplan-Meier法计算无复发生存期（RFS）。进行单变量和多变量Cox比例风险建模以获得复发风险比（HR）。
94例初发局限性疾病患者中，有21例复发：4例局部复发，14例远处复发，3例混合复发。5年和10年RFS分别为75.6%和70.5%。多变量分析包括诊断时年龄和肿瘤大小，新辅助化疗（NAC）后肿瘤坏死<95%（HR 14.3，P = 0.028）和NAC期间放射学肿瘤大小变化（每1%大小变化减少HR 1.04，P = 0.032）是复发的独立预测因素。在发生远处复发的患者中，82%有肺转移，53%肺转移是唯一可识别的疾病部位。
NAC后病理或放射学反应不佳可预测局限性尤因肉瘤患者复发。化疗后肿瘤大小缩小不理想为未行根治性切除的患者提供了风险分层方法。孤立性肺复发是常见事件。
