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Rabdomiosarcoma genitourinario en la infancia, nuestra experiencia. [artículo]

Por: Morante Valverde, Rocío [Cirugía Pediátrica] | Cabezalí Barbancho, Daniel [Cirugía Pediátrica] | Moreno Zegarra, Cecilia [Cirugía Pediátrica] | González Herrero, Marina [Cirugía Pediátrica] | Tordable Ojeda, Cristina [Cirugía Pediátrica] | López Vázquez, Francisco [Cirugía Pediátrica] | Aransay Bramtot, Adolfo [Cirugía Pediátrica] | Gómez Fraile, Andrés [Cirugía Pediátrica].
Colaborador(es): Servicio de Cirugía Pediátrica.
Tipo de material: materialTypeLabelArtículoEditor: Cirugía pediátrica : órgano oficial de la Sociedad Española de Cirugía Pediátrica, 2014Descripción: 27(2):53-56.Recursos en línea: Solicitar documento Resumen: Introduction: 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. Material and methods: 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. Results: 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. Conclusions: 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.
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Revisión Revisión PC16297 (Navegar estantería) Disponible

Formato Vancouver:
Morante Valverde R, Cabezalí Barbanco D, Moreno Zegarra C, González Herrero M, Tordable Ojeda C, López Vázquez F et al. Rabdomiosarcoma genitourinario en la infancia, nuestra experiencia. Cir Pediatr. 2014 Apr 15;27(2):53-56.

PMID: 27775271

Contiene 8 referencias

Introduction: 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.
Material and methods: 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.
Results: 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.

Conclusions: 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.

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