Estado del arte: tumores de cresta neural, neuroblastoma fisiopatología, diagnóstico y tratamiento
Resumen
El neuroblastoma es el tumor sólido extracraneal más frecuente en la infancia, con incidencia de 650 casos al año en los Estados Unidos de America y 100 casos por año en Italia y España. Se trata de un tumor que evoluciona de forma variable, desde la regresión espontánea hasta un comportamiento muy maligno, sobre todo en niños en edades mayores y con la existencia de enfermedad diseminada. Los signos y síntomas de presentación del neuroblastoma reflejan la localización del tumor y debido a las múltiples presentaciones clínicas, puede confundirse con gran variedad de patologías. Su tratamiento incluye la cirugía, la quimioterapia, la radioterapia y la terapia inmunológica. El papel de cada uno se determina anticipando el comportamiento clínico del tumor en cada caso, considerando la edad, el estadio y determinados parámetros biológicos. En los últimos 10 años, la estratificación por grupos de riesgo de los pacientes con neuroblastoma, basada en el análisis de un panel amplio de variables clínicas y biológicas, entre las que destacan la edad, el estadio y la amplificación del N-MYC, ha permitido un diagnóstico temprano y nuevas pautas para una mejoría importante en su tratamiento. El tratamiento del neuroblastoma se diseña hoy en día según grupos de riesgo, que se definen por parámetros clínicos y biológicos cada vez más sofisticados. El mejor conocimiento de la biología del neuroblastoma ha permitido distinguir entre aquellos tumores que pueden curarse con un tratamiento mínimo y los que requieren de un tratamiento multidisciplinario y complejo para tener posibilidades de curación. Es fundamental realizar al diagnóstico temprano con indicaciones de estadio y desarrollo de una terapia oportuna.Descargas
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