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• W2569887594 abstract "Rhabdomyosarcoma (RSM) becomes the most common tumour of the soft tissues during the paediatric age. It represents among 2-3% of child tumours. The genital-urinary location is the second most common location, only after head and neck. The treatment is usually medical, being the surgery a mere contribution, except for the cases in which the situation is not under control, when very aggressive surgery is necessary. The aim of this study is to analyse the cases of genial-urinary RMS that have been treated in our centre and the role that surgery has in their treatment.Retrospective study of 20 patient (7 girls and 13 boys) with a median age of 24 months (range from 1 month to 12 years) with RMS in the aurochs-genial tract who have been treated in our hospital from 1990 to 2012. The variables described are demographic, location of the primary tumour, state at diagnosis, received treatment, both medical and surgical, with greater emphasis on the kind of surgery applied and monitoring in terms of survival.The location of the primary tumour was: bladder (6), paratesticular (5), vagina (3) retroperitoneal space (3), lesser pelvis (2) and prostate (1). All of them received medical treatment with chemotherapy and radiotherapy following International Society of Pediatric Oncology protocol after diagnostic biopsy. Surgery, which was always used as help, was: reappraisal of biopsy (1), orchiectomy (5), tumoral resection (8) and radical surgery (cystoprostatectomy or pelvic exenteration) in 6 patients. There were 3 deaths, 2 because of the evolution of the disease and 1 because of postoperative sepsis. The survival rate is 80% with a median follow - up of 14 years.The RMS is the most common tumour of soft tissues in childhood and the genital-urinary location is the second most common after the parameningeal one. The treatment is multidisciplinary and the surgery has a contributing role when there is no answer to the medical treatment or when there is a residual tumour even if some patients do not respond to medical treatment and they need a radical surgery for recovery.El rabdomiosarcoma (RMS) constituye el tumor de tejidos blandos más frecuente en la edad pediátrica, representando el 2-3% de los tumores infantiles. La localización genitourinaria es la segunda en frecuencia tras la cabeza y cuello. El tratamiento suele ser médico, quedando la cirugía como coadyuvante, excepto en casos no controlados en que se precisan cirugías muy agresivas. El objetivo del estudio es analizar los casos de RMS de localización genitourinaria tratados en nuestro Centro y el papel que la cirugía tiene en su tratamiento.Estudio retrospectivo de 20 pacientes (7 niñas y 13 niños) con una mediana de edad de 24 meses (rango de 1 mes a 12 años) con RMS del tracto urogenital tratados en nuestro Hospital desde 1990 hasta 2012. Se describen variables demográficas, localización del tumor primario, estadio al diagnóstico, tratamiento recibido, tanto médico como quirúrgico, con especial atención al tipo de cirugía realizada y seguimiento en términos de supervivencia.La localización del tumor primario fue: vejiga (6), paratesticular (5), vagina (3), retroperitoneo (3), pelvis menor (2) y próstata (1). Todos recibieron tratamiento médico con quimioterapia y radioterapia según protocolo de la Sociedad Internacional de Oncología Pediátrica (SIOP) previa biopsia diagnóstica. La cirugía, practicada en todos los casos como coadyuvante fue: reevaluación por biopsia (1), orquiectomía (5), resección tumoral (8) y cirugía radical (cistoprostatectomía o exanteración pélvica) en 6 pacientes. Hubo 3 fallecimientos, 2 por progresión de la enfermedad y 1 por sepsis postoperatoria. Los 17 restantes están vivos, lo que supone una supervivencia del 80% con una mediana de seguimiento de 14 años.El RMS es el tumor de tejidos blandos más frecuente en la infancia y la localización genitourinaria la segunda en frecuencia tras las parameníngeas. El tratamiento es multidisciplinar y la cirugía tiene un papel coadyuvante en casos de no respuesta al tratamiento médico o de tumor residual aunque hay pacientes que no responden al tratamiento médico y precisan de cirugía radical para su curación." @default.
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• W2569887594 date "2014-04-15" @default.
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• W2569887594 title "[Genital-urinary rhabdomyosarcoma in childhood: our experience]." @default.
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