Craneofaringioma en pediatría : diagnóstico y tratamiento.
Craniopharyngioma in pediatrics: diagnosis and treatment
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Los craneofaringiomas (CF) son tumores raros de baja malignidad histológica, que surgen a lo largo del conducto craneofaríngeo. Los dos subtipos histológicos, craneofaringioma adamantinomatoso (CFA) y papilar (CFP), difieren en su origen y distribución por edad. Los CFA se diagnostican con un pico de incidencia bimodal (5–15 años y 45–60 años), mientras que los CFP se presentan exclusivamente en adultos, principalmente en la quinta y sexta décadas de la vida. Los CFA son generados por mutaciones somáticas en CTNNB1, codificador de β-catenina y son predominantemente de aspecto quístico. Los CFP albergan mutaciones somáticas en BRAF V600E y son típicamente tumores sólidos. Las manifestaciones clínicas de hipertensión endocraneana, compromiso visual o deficiencias endocrinas, sirven de alerta para solicitar imágenes. El tratamiento comprende generalmente neurocirugía y radioterapia (RT); la quimioterapia intraquística se usa en el CFA monoquístico. Aunque la supervivencia a largo plazo es alta, con frecuencia se ven afectadas la calidad de vida y la función neuropsicológica, debido a la proximidad anatómica al quiasma óptico, el hipotálamo y la glándula pituitaria. La afectación hipotalámica por el tumor y las lesiones secundarias al tratamiento, con frecuencia resultan en obesidad hipotalámica y déficits neuropsicosociales.
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