Craneofaringioma en pediatría : diagnóstico y tratamiento.
Titulo:

Craneofaringioma en pediatría : diagnóstico y tratamiento.
.

Sumario:

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... Ver más

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Revista Colombiana de Hematología y Oncología

2256-2877

2256-2915

Cabrera Bernal, Edgar

ASOCIACION COLOMBIANA DE HEMATOLOGIA Y ONCOLOGIA

10.51643/22562915.112

7

2020-08-01

64

74

Colombia

pediatría

clasificación

fisiopatología

diagnóstico

diagnóstico por imagen

cirugía

radioterapia

tratamiento farmacológico

craneofaringioma

Edgar Cabrera Bernal - 2020

Esta obra está bajo una licencia internacional Creative Commons Atribución-NoComercial-SinDerivadas 4.0.

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spelling Craneofaringioma en pediatría : diagnóstico y tratamiento.
Craniopharyngioma in pediatrics: diagnosis and treatment
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.
Craniopharyngiomas (CP) are rare tumors of low histological malignancy that arise along the craniopharyngeal duct. The two histological subtypes, adamantinomatous craniopharyngioma (ACP) and papillary (PCP), differ in origin and distribution by age. ACPs are diagnosed with a bimodal incidence peak (5–15 years and 45–60 years), while PCPs occur exclusively in adults, mainly in the fifth and sixth decades of life. ACPs are generated by somatic mutations in CTNNB1, encoder of β-catenin and are predominantly cystic in appearance. PCPs have somatic mutations in BRAF V600E and are typically solid tumors. The clinical manifestations of endocranial hypertension, visual compromise or endocrine deficiencies should alert us to request images. The treatment generally includes neurosurgery and radiotherapy (RT); Intracystic chemotherapy is used in monocystic ACP. Although long-term survival is high, quality of life and neuropsychological function are frequently affected, due to the anatomical proximity to the optic chiasma, the hypothalamus and the pituitary gland. Hypothalamic involvement by the tumor and injuries secondary to treatment often result in hypothalamic obesity and neuropsicosocial deficits.
Cabrera Bernal, Edgar
pediatría
clasificación
fisiopatología
diagnóstico
diagnóstico por imagen
cirugía
radioterapia
tratamiento farmacológico
craneofaringioma
pediatrics
classification
physiopathology
diagnosis
diagnostic imaging
surgery
radiotherapy
drug therapy
craniopharyngioma
7
2
Núm. 2 , Año 2020 : Julio - Diciembre
Artículo de revista
Journal article
2020-08-01T00:00:00Z
2020-08-01T00:00:00Z
2020-08-01
application/pdf
Asociación Colombiana de Hematología y Oncología (ACHO)
Revista Colombiana de Hematología y Oncología
2256-2877
2256-2915
https://revista.acho.info/index.php/acho/article/view/112
10.51643/22562915.112
https://doi.org/10.51643/22562915.112
spa
https://creativecommons.org/licenses/by-nc-nd/4.0
Edgar Cabrera Bernal - 2020
Esta obra está bajo una licencia internacional Creative Commons Atribución-NoComercial-SinDerivadas 4.0.
64
74
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institution ASOCIACION COLOMBIANA DE HEMATOLOGIA Y ONCOLOGIA
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collection Revista Colombiana de Hematología y Oncología
title Craneofaringioma en pediatría : diagnóstico y tratamiento.
spellingShingle Craneofaringioma en pediatría : diagnóstico y tratamiento.
Cabrera Bernal, Edgar
pediatría
clasificación
fisiopatología
diagnóstico
diagnóstico por imagen
cirugía
radioterapia
tratamiento farmacológico
craneofaringioma
pediatrics
classification
physiopathology
diagnosis
diagnostic imaging
surgery
radiotherapy
drug therapy
craniopharyngioma
title_short Craneofaringioma en pediatría : diagnóstico y tratamiento.
title_full Craneofaringioma en pediatría : diagnóstico y tratamiento.
title_fullStr Craneofaringioma en pediatría : diagnóstico y tratamiento.
title_full_unstemmed Craneofaringioma en pediatría : diagnóstico y tratamiento.
title_sort craneofaringioma en pediatría : diagnóstico y tratamiento.
title_eng Craniopharyngioma in pediatrics: diagnosis and treatment
description 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.
description_eng Craniopharyngiomas (CP) are rare tumors of low histological malignancy that arise along the craniopharyngeal duct. The two histological subtypes, adamantinomatous craniopharyngioma (ACP) and papillary (PCP), differ in origin and distribution by age. ACPs are diagnosed with a bimodal incidence peak (5–15 years and 45–60 years), while PCPs occur exclusively in adults, mainly in the fifth and sixth decades of life. ACPs are generated by somatic mutations in CTNNB1, encoder of β-catenin and are predominantly cystic in appearance. PCPs have somatic mutations in BRAF V600E and are typically solid tumors. The clinical manifestations of endocranial hypertension, visual compromise or endocrine deficiencies should alert us to request images. The treatment generally includes neurosurgery and radiotherapy (RT); Intracystic chemotherapy is used in monocystic ACP. Although long-term survival is high, quality of life and neuropsychological function are frequently affected, due to the anatomical proximity to the optic chiasma, the hypothalamus and the pituitary gland. Hypothalamic involvement by the tumor and injuries secondary to treatment often result in hypothalamic obesity and neuropsicosocial deficits.
author Cabrera Bernal, Edgar
author_facet Cabrera Bernal, Edgar
topicspa_str_mv pediatría
clasificación
fisiopatología
diagnóstico
diagnóstico por imagen
cirugía
radioterapia
tratamiento farmacológico
craneofaringioma
topic pediatría
clasificación
fisiopatología
diagnóstico
diagnóstico por imagen
cirugía
radioterapia
tratamiento farmacológico
craneofaringioma
pediatrics
classification
physiopathology
diagnosis
diagnostic imaging
surgery
radiotherapy
drug therapy
craniopharyngioma
topic_facet pediatría
clasificación
fisiopatología
diagnóstico
diagnóstico por imagen
cirugía
radioterapia
tratamiento farmacológico
craneofaringioma
pediatrics
classification
physiopathology
diagnosis
diagnostic imaging
surgery
radiotherapy
drug therapy
craniopharyngioma
citationvolume 7
citationissue 2
citationedition Núm. 2 , Año 2020 : Julio - Diciembre
publisher Asociación Colombiana de Hematología y Oncología (ACHO)
ispartofjournal Revista Colombiana de Hematología y Oncología
source https://revista.acho.info/index.php/acho/article/view/112
language spa
format Article
rights https://creativecommons.org/licenses/by-nc-nd/4.0
Edgar Cabrera Bernal - 2020
Esta obra está bajo una licencia internacional Creative Commons Atribución-NoComercial-SinDerivadas 4.0.
info:eu-repo/semantics/openAccess
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