Tumor teratoide rabdoide atípico en un adulto joven.
Atypical teratoid rhabdoid tumor in an adult patient.
Contenido principal del artículo
Se presenta el caso de un hombre de 28 años con pérdida progresiva de la agudeza visual y cefalea asociada a polidipsia, poliuria e hipersomnia. La resonancia magnética cerebral mostró una masa heterogénea localizada a nivel selar/supraselar con realce intenso después de la administración del contraste. Se realizó una resección parcial con patología que evidenció una lesión tumoral de apariencia rabdoide con pérdida de la expresión del INI-1. Estos hallazgos fueron compatibles con un tumor teratoide/rabdoide atípico tratado con teleterapia y el esquema de quimioterapia ICE con adecuada respuesta. Se discute el resultado después de cinco meses de tratamiento en espera de la administración de quimioterapia con intensidad de dosis más rescate con progenitores obtenidos de sangre periférica. También se presenta una revisión de los datos referentes al manejo de esta neoplasia, al igual que de los mecanismos moleculares implicados en su fisiopatología.
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