Tumor neuroectodérmico gastrointestinal maligno (GNET) : a propósito de un caso.
Gastrointestinal neuroectodermal tumor (GNET) : a case.
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El tumor neuroectodérmico gastrointestinal maligno (GNET) es una entidad extremadamente rara recientemente descrita en la literatura médica y que también es conocida con el nombre de tumor similar al sarcoma de células claras en el tracto gastrointestinal (CCSLGT) por su similitud morfológica y molecular con el sarcoma de células claras de tendones y aponeurosis (CCS), pero, dada la presencia de rearreglos de gen EWSR1 y la diferenciación neuroectodérmica, se ha considerado por algunos autores más apropiado el término GNET. Es una enfermedad con pobre pronóstico, alta tasa de recurrencia y casi siempre metastásica desde el diagnóstico, lo que contrasta con el comportamiento relativamente indolente del CSS. En nuestro conocimiento, existen menos de 50 casos reportados de esta patología y a la fecha este sería el primero con primario localizado en hígado y con sobrevida libre de recaída prolongada luego de manejo quirúrgico adecuado y quimioterapia adyuvante.
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- http://clinicaltrials.gov/ct2/show/NCT01524926