Çocukluk Çağı Ewing Sarkomlu Hastaların Klinik Özellikleri ve Tedavilerinin İncelenmesi

Abstract

Gül G., Examination of Clinical Features and Treatments of Childhood Ewing Sarcoma Patients, Hacettepe University Faculty of Medicine, MD Thesis, Department of Pediatrics. Ankara, 2024 Background and Aims: Five-year survival rates have increased to 70-80% in children with Ewing Sarcoma. This study aimed to examine the clinical characteristics and treatment results of children with Ewing sarcoma. Methods: 175 patients with Ewing sarcoma (ES) diagnosed between January, 2000 and December, 2022 were included in this analysis. The clinical features and outcomes were recorded from patient files and hospital information system retrospectively. Results: The median age at diagnosis was 10.6 years (0.4-17.6 years), female/male ratio was 1.3. The most common symptoms were pain (62.3%) and mass (47,4%). The common sites were extremities, trunk and pelvic in 39.4%, 29.7% and 22.3% of patients, respectively. Metastatic disease was present in 34.8% children. Treatment regimens included the CDCV (MMR, modified malignant rhabdoid tumor protocol; cisplatin, cyclophosphamide, adriamycin, vincristine) in 26, PIAV (cisplatin, ifosfamide, adriamycin, vincristine) in 63 and Euro-Ewing 99 (EE99) in 86 children. Radiotherapy was given to 102 children and 118 had a major tumor resection. The five-year OS and EFS were 57.4% & 45.8% for the whole group. The five-year OS and EFS rates were 57.7% & 46.2%; 49.2% & 38.1%; 63.4% & 52.3% in children receiving CDCV, PIAV and EE99 respectively (p=0.1). The five-year OS and EFS rates were 36.1% & 25% vs 69.6% & 57.5% in children with metastatic vs non-metastatic disease (p<0.05). The OS and EFS rates were 71.9% & 56.1%; 59.5% & 48.3%; 53.8% & 51.5%; 41.7% & 24.4%; 85.7% & 75.0% in children with tumors located in upper extremity, lower extremity, trunk, pelvic and cranial sites, respectively (p=0.04). The five-year OS for refractory and relapsed disease was 35.2% and 24.5%, respectively. Conclusions: Treatment results show the progress made in achieving better survival rates over the years with the latest treatment approaches in our department. The tumor’s location, volume, and stage and extent of disease have prognostic significance for survival rates. Keywords: Ewing sarcoma, childhood, chemotherapy, surgery, radiotherapy