Abstract
Simple SummaryThe lung is the most frequent site of metastasis in Ewing sarcoma, the second most common bone cancer affecting children, adolescents and young adults. The five-year overall survival of patients with isolated lung metastasis is approximately 50% after multimodal treatments including chemotherapy, surgery and radiotherapy. This retrospective study aimed to investigate the feasibility and the predictors of survival in 68 Ewing sarcoma patients with lung metastases who received high-dose chemotherapy with busulfan and melphalan, followed by reduced dose whole-lung irradiation, as part of two prospective and consecutive treatment protocols. This combined treatment strategy is feasible and might contribute to the disease control in lung metastatic Ewing sarcoma with responsive disease. Furthermore, the results of this study provide support to explore the treatment stratification for lung metastatic Ewing sarcoma based on the histological response of the primary tumor.Purpose: To analyze toxicity and outcome predictors in Ewing sarcoma patients with lung metastases treated with busulfan and melphalan (BU-MEL) followed by whole-lung irradiation (WLI). Methods: This retrospective study included 68 lung metastatic Ewing Sarcoma patients who underwent WLI after BU-MEL with autologous stem cell transplantation, as part of two prospective and consecutive treatment protocols. WLI 12 Gy for <14 years old and 15 Gy for ≥14 years old patients were applied at least eight weeks after BU-MEL. Toxicity, overall survival (OS), event-free survival (EFS) and pulmonary relapse-free survival (PRFS) were estimated and analyzed. Results: After WLI, grade 1–2 and grade 3 clinical toxicity was reported in 16.2% and 5.9% patients, respectively. The five-year OS, EFS and PRFS with 95% confidence interval (CI) were 69.8% (57.1–79.3), 61.2% (48.4–71.7) and 70.5% (56.3–80.8), respectively. Patients with good histological necrosis of the primary tumor after neoadjuvant chemotherapy showed a significant decreased risk of pulmonary relapse or death compared to patients with poor histological necrosis. Conclusions: WLI at recommended doses and time interval after BU-MEL is feasible and might contribute to the disease control in Ewing sarcoma with lung metastases and responsive disease. Further studies are needed to explore the treatment stratification based on the histological response of the primary tumor.
Highlights
Ewing sarcoma (ES) is the second most common bone tumor affecting children, adolescents and young adults [1]
Data from sixty-eight lung metastatic ES patients treated with busulfan and melphalan (BU-MEL) followed by whole-lung irradiation (WLI) were collected
All patients resulted evaluable for the analysis
Summary
Ewing sarcoma (ES) is the second most common bone tumor affecting children, adolescents and young adults [1]. 25% of patients with ES have metastatic disease at diagnosis and the lung is the most frequent site of metastasis [2,3]. The current five-year OS for patients with localized disease is 65% to 75% [7,8,9,10], patients with metastases have a long-term survival less than 35% [3,11], except for those with isolated lung metastasis in whom the five-year OS is approximately 50% [12,13,14]. In international guidelines WLI is strongly recommended for metastatic disease to the lungs at doses of 15 or 18 Gray (Gy), according to the age of the patient [20]. Other studies suggested that the impact on the outcome of the different treatment modalities, including WLI, needs to be better defined [21,22,23]
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