Ewing sarcoma (ES) is a rare and aggressive disease that requires multidisciplinary treatment with the use of chemotherapy, radiotherapy, and surgery. Our retrospective study aimed to analyze the prognostic factors and treatment results in different age groups of patients. Between 1998 and 2018, 569 patients with ES were treated in two referral centers. The patients were divided into four age groups (≤10 years; 11–18 years; 19–25, and >25). The treatment results and prognostic factors were assessed for each group. For statistical analyses, we used the Chi2 test, the Kaplan–Meier estimator with a log-rank test, and the multivariate Cox model. Five-year overall survival (OS) rate was 56%. In the age subgroups: ≤10 years, 11–18 years, 19–25 years, and >25 years, the 5-year OS rates were 75%, 58%, 41%, and 52%, respectively. Favorable prognostic factors: female gender (p = 0.024), non-axial localization (p = 0.005), VIDE regimen (p < 0.001), and surgery as a local treatment (p < 0.001) dominated in the group ≤10 years. In multivariate analysis, male (HR = 1.53), axial localization (HR = 1.46), M1 status at presentation (HR = 2.64), and age > 10 years (HR = 2.29) were associated with shorter OS. The treatment results in ES are significantly better in children aged ≤10 years; the challenge is to provide therapy for adolescents and young adults. The diagnostics and treatment of ES patients must be provided in referral centers.

Introduction Ewing sarcoma (ES) is a highly aggressive malignancy of bone and soft tissues characterized by the presence of a genetic fusion involving the EWSR1 gene. More than one-third of patients develop distant metastases, which are associated with unfavorable prognosis. Knowledge about the...

Ewing sarcoma is a group of low-differentiated, high-grade, small-cell tumours. It is the third most common malignancy among primary bone tumours in adults. The prognosis of Ewing sarcoma is bad, and very often at the time of diagnosis the disease is highly advanced. For patients with localized disease, who have no metastases diagnosed at baseline, it is necessary to use combined treatment. It consists of induction chemotherapy (12–18 weeks) + local treatment (surgery ± radiotherapy or radiotherapy), and is followed by adjuvant consolidation chemotherapy. The whole treatment takes about 48–52 weeks. Advances in multidisciplinary treatment, including combined chemotherapy, surgery and radiotherapy have resulted in a significant improvement in patients’ quality of life and prognosis. Clinical trials are currently underway and new treatment standards are being developed. Ewing sarcoma is a group of low-differentiated, high-grade, small-cell tumours. It is the third most common malignancy among primary bone tumours in adults. The prognosis of Ewing sarcoma is bad, and very often at the time of diagnosis the disease is highly advanced. For patients with localized disease, who have no metastases diagnosed at baseline, it is necessary to use combined treatment. It consists of induction chemotherapy (12–18 weeks) + local treatment (surgery ± radiotherapy or radiotherapy), and is followed by adjuvant consolidation chemotherapy. The whole treatment takes about 48–52 weeks. Advances in multidisciplinary treatment, including combined chemotherapy, surgery and radiotherapy have resulted in a significant improvement in patients’ quality of life and prognosis. Clinical trials are currently underway and new treatment standards are being developed.

Encorafenib and binimetinib were registered in 2018 for the treatment of adult patients with unresectable or metastatic melanoma with a BRAF V600 mutation. The results of the phase III study (Columbus) are very promising. Median PFS for patients who have received this treatment was 14.9 months, and the median OS was 33.6 months. The reduction of toxicity is the reason for the unique pharmacokinetic profile of this therapy. Knowledge about the adverse evets is important in the context of optimizing and individualizing treatment. Encorafenib and binimetinib were registered in 2018 for the treatment of adult patients with unresectable or metastatic melanoma with a BRAF V600 mutation. The results of the phase III study (Columbus) are very promising. Median PFS for patients who have received this treatment was 14.9 months, and the median OS was 33.6 months. The reduction of toxicity is the reason for the unique pharmacokinetic profile of this therapy. Knowledge about the adverse evets is important in the context of optimizing and individualizing treatment.