부산대학교 도서관

Simple Summary: Ewing sarcoma (EwS) is a malignant bone and soft tissue cancer that requires intensive treatment with multiple chemotherapies and either surgery, irradiation, or both as local therapy. For most survivors of EwS, long-term sequelae such as secondary malignant neoplasms (SMNs) other than EwS are concerning. Few studies suggest that SMNs after EwS are a rare but serious event. Comprehensive data are lacking. We reviewed consecutive EwS trials from the Cooperative Ewing Sarcoma Study (CESS) group to evaluate the features of SMNs in EwS patients. Our analysis revealed 101 cases of SMNs in 96 EwS patients. Solid SMNs were detected more frequently than hematologic SMNs, in 55.2% versus 44.8%. The latency between EwS diagnosis and SMN occurrence was longer for solid SMNs (median: 8.4 years) than for hematologic SMNs (median: 2.4 years) (p < 0.001). The survival rate after SMNs was 0.49, with solid SMNs having a significantly better prognosis. Our results confirm the need for a structured follow-up system. Ewing sarcoma (EwS) represents highly aggressive bone and soft tissue tumors that require intensive treatment by multi-chemotherapy, surgery and/or radiotherapy. While therapeutic regimens have increased survival rates, EwS survivors face long-term sequelae that include secondary malignant neoplasms (SMNs). Consequently, more knowledge about EwS patients who develop SMNs is needed to identify high-risk patients and adjust follow-up strategies. We retrospectively analyzed data from 4518 EwS patients treated in five consecutive EwS trials from the Cooperative Ewing Sarcoma Study (CESS) group. Ninety-six patients developed SMNs after primary EwS, including 53 (55.2%) with solid tumors. The latency period between EwS and the first SMN was significantly longer for the development of solid SMNs (median: 8.4 years) than for hematologic SMNs (median: 2.4 years) (p < 0.001). The cumulative incidence (CI) of SMNs in general increased over time from 0.04 at 10 years to 0.14 at 30 years; notably, the specific CI for hematologic SMNs remained stable over the different decades, whereas for solid SMNs it gradually increased over time and was higher for metastatic patients than in localized EwS patients (20 years: 0.14 vs. 0.06; p < 0.01). The clinical characteristics of primary EwS did not differ between patients with or without SMNs. All EwS patients received multi-chemotherapy with adjuvant radiotherapy in 77 of 96 (80.2%) patients, and the use of radiation doses ≥ 60 Gy correlated with the occurrence of SMNs. The survival rate after SMNs was 0.49, with a significantly better outcome for solid SMNs compared with hematologic SMNs (3 years: 0.70 vs. 0.24, respectively; p < 0.001). The occurrence of SMNs after EwS remains a rare event but requires a structured follow-up system because it is associated with high morbidity and mortality. [ABSTRACT FROM AUTHOR]