Tumor neuroectodérmico gastrointestinal maligno (GNET) : a propósito de un caso.

Gastrointestinal neuroectodermal tumor (GNET) : a case.

Contenido principal del artículo

Diego Mauricio González Ramírez
Juan Carlos Velásquez
Yenni Andrea Quevedo Morales
Carlos Eduardo Bonilla
Ricardo Elías Bruges

Resumen

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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Referencias (VER)

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http://clinicaltrials.gov/ct2/show/NCT01524926