Sarcoma de Ewing, análisis de supervivencia a los 6 años con terapia multidisciplinar / Ewing sarcoma, analysis of survival at 6 years with multidisciplinary therapy

Objetivo: El objetivo de este estudio es presentar nuestra serie de casos de sarcoma de Ewing y los datos de supervivencia obtenidos a medio plazo utilizando un protocolo de terapia multidisciplinar. Material, método y resultados: Cuarenta y un sarcomas de Ewing fueron diagnosticados, tratados y evolucionados en nuestro centro entre 2004 y 2009 con una media de edad de 18,29 años. Hasta un 78% correspondieron a Ewing óseo siendo el fémur la localización más frecuente. El 68% presentó un estadio localizado en el momento del diagnóstico. Al final del seguimiento la supervivencia no llegó al 40% de los pacientes, falleciendo la mayoría en los primeros 5 años de seguimiento. Discusión: En España, el sarcoma de Ewing es el tumor óseo maligno primario más frecuente en la infancia, por delante del osteosarcoma. Su tasa de supervivencia ha aumentado mucho en los últimos 40 años, mejoría atribuible fundamentalmente al uso agresivo de la quimioterapia y al tratamiento mutidisciplinar, pero su pronóstico sigue siendo muy pobre sobre todo en aquellos que presentan metástasis al diagnóstico, principal factor pronóstico adverso. Dada su alta mortalidad, son muchos los autores que lo consideran como una enfermedad diseminada desde el principio, con micrometástasis no detectables que son las que condicionan la supervivencia final. Conclusiones: El diagnóstico precoz y el tratamiento multidisciplinar en centros de referencia son las mejores estrategias con las que contamos en la actualidad para proporcionarles a los pacientes las máximas posibilidades de curación de esta enfermedad

Purpose: The purpose of this study is to present our series of Ewing sarcoma cases and the survival data obtained in the medium term, using a multidisciplinary therapy protocol. Material, methods and results: Forty-one Ewing sarcomas were diagnosed, treated and followed-up in our hospital between 2004 and 2009 with an average age of 18.29 years. Seventy-eight percent were to Ewing sarcoma of the bone, the femur being the most frequent location. Sixty-eight percent had a localized stage at the time of diagnosis. At the end of follow-up, 40% of the patients did not survive, most died within the first 5 years of follow-up. Discussion: In Spain, Ewing sarcoma is the most common primary malignant bone tumour in childhood, ahead of osteosarcoma. Its survival rate has increased greatly in the last 40 years, improvement attributable mainly to the aggressive use of chemotherapy and to multidisciplinary treatment, but its prognosis remains very poor, especially for those with metastasis at diagnosis, the main adverse prognostic factor. Because of its high mortality, many authors consider it a disseminated disease from the beginning, with non- detectable micrometastasis that condition final survival. Conclusions: Early diagnosis and multidisciplinary therapy in referral centres are the best strategies currently available to us to provide these patients the maximum possibilities of cure of this disease

Ewing sarcoma, analysis of survival at 6 years with multidisciplinary therapy. / Sarcoma de Ewing, análisis de supervivencia a los 6 años con terapia multidisciplinar.

PURPOSE: The purpose of this study is to present our series of Ewing sarcoma cases and the survival data obtained in the medium term, using a multidisciplinary therapy protocol. MATERIAL, METHODS AND RESULTS: Forty-one Ewing sarcomas were diagnosed, treated and followed-up in our hospital between 2004 and 2009 with an average age of 18.29 years. Seventy-eight percent were to Ewing sarcoma of the bone, the femur being the most frequent location. Sixty-eight percent had a localized stage at the time of diagnosis. At the end of follow-up, 40% of the patients did not survive, most died within the first 5 years of follow-up. DISCUSSION: In Spain, Ewing sarcoma is the most common primary malignant bone tumour in childhood, ahead of osteosarcoma. Its survival rate has increased greatly in the last 40 years, improvement attributable mainly to the aggressive use of chemotherapy and to multidisciplinary treatment, but its prognosis remains very poor, especially for those with metastasis at diagnosis, the main adverse prognostic factor. Because of its high mortality, many authors consider it a disseminated disease from the beginning, with non- detectable micrometastasis that condition final survival. CONCLUSIONS: Early diagnosis and multidisciplinary therapy in referral centres are the best strategies currently available to us to provide these patients the maximum possibilities of cure of this disease.