Abstract
Ewing sarcoma, a rare malignancy of childhood and adolescence, has become a model of progress in diagnosis and treatment through long-standing research efforts in multinational clinical trials. The aim of this study is to present the specific diagnostic and therapeutic approach of Ewing sarcoma in children and adolescents. Materials and method. A retrospective and prospective analysis of all Ewing sarcoma treated in a large referral center – the Department of Pediatric and Orthopedic Surgery, Maria Sklodowska Curie Emergency Hospital for Children, between 2005 and 2012 is presented. A total of 28 patients were identified, 19 boys and 9 girls, with a male to female ratio of 2.11:1. Diagnosis was based on the result of the histopathological examination of tumor biopsy. Results. The mean age of the patients was 12.3 years (range 3-19 years) and the mean tumor volume was 197.96 cm3 (range 8-1,200 cm3). 8/28 patients (28.57%) had metastatic disease at diagnosis, mainly in the lungs (7 cases). Chemotherapy was administred to 26 patients (92.85%). Local therapy consisted of surgery in all patients and of surgery combined with radiation, in 5 patients. After a mean follow-up of 51.5 months, 9 patients have died, 17 (60.71%) patients are alive and free of disease and 2 patients are alive with disease. Conclusions. The management of a child or adolescent with Ewing sarcoma is best carried out in a specialized center under the care of a multidisciplinary team, in order to obtain the best outcome for the patient. Ewing sarcoma has a high mortality rate in Romania, especially because of late diagnosis.
Highlights
Ewing sarcoma (ES), a rare malignancy of childhood and adolescence, has become a model of advances in diagnosis and treatment through longstanding research efforts in multinational clinical trials
The time until diagnosis was long; in 17.85% (5/28) it was less than 3 months, in 50% (14/28) it was between 3-6 months and in 32.14% (9/28) was more than 6 months
The most common presenting symptom was local pain, frequently associated with trauma, followed by swelling, functional disability and pathologic fracture in four cases
Summary
Ewing sarcoma (ES), a rare malignancy of childhood and adolescence, has become a model of advances in diagnosis and treatment through longstanding research efforts in multinational clinical trials. Ewing sarcoma (ES) is the second most common primary bone tumor in children and adolescents, only exceeded in prevalence by osteosarcoma; the overall annual incidence is 2.93 cases per 1,000,000 people [3]. It is rare in patients younger than 5 or older than 30, with 95% of cases reported between the ages of 4 and 25 years and has a slight male predilection (M:F 1.5:1) [1,4]. It occurs in the spine or in the small bones of the feet or hands. [3]
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