Modern treatment of craniopharyngioma to improve outcomes: evidence of a change of paradigm

Background: Craniopharyngiomas, rare primary brain tumors of the pituitary-hypothalamic axis, frequently result in sicubstantial morbidity, including compromised quality of life, vision impairment, hypothalamic and endocrine dysfunction, and neuroendocrine disturbances. Of particular importance is t...

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Autores: González-Gallego, Carlos, Molina, Pedro, Hostalot, Cristina, Oliva, Anna, Blanco, Alberto, Puyalto de Pablo, Paloma, Comas, Silvia, Carrato, Cristina, Valassi, Elena, Puig-Domingo, Manel
Tipo de recurso: artículo
Fecha de publicación:2025
País:España
Institución:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
Repositorio:Recercat. Dipósit de la Recerca de Catalunya
OAI Identifier:oai:recercat.cat:20.500.12328/5061
Acceso en línea:http://hdl.handle.net/20.500.12328/5061
https://dx.doi.org/10.1007/s12020-025-04216-9
Access Level:acceso abierto
Palabra clave:Inhibidors de BRAF-MEK
Craniofaringioma papil·lar
Craniofaringioma adamantinomatós
Mutació BRAF V600E
Wnt/beta-catenina
Tractament dirigit
Inhibidores de BRAF-MEK
Craneofaringioma papilar
Craneofaringioma adamantinomatoso
Mutación BRAF V600E
Tratamiento dirigido
BRAF-MEK inhibitors
Papillary craniopharyngioma
Adamantinomatous craniopharyngioma
BRAF V600E mutation
Wnt/beta-catenin
Targeted treatment
61
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spelling Modern treatment of craniopharyngioma to improve outcomes: evidence of a change of paradigmGonzález-Gallego, CarlosMolina, PedroHostalot, CristinaOliva, AnnaBlanco, AlbertoPuyalto de Pablo, PalomaComas, SilviaCarrato, CristinaValassi, ElenaPuig-Domingo, ManelInhibidors de BRAF-MEKCraniofaringioma papil·larCraniofaringioma adamantinomatósMutació BRAF V600EWnt/beta-cateninaTractament dirigitInhibidores de BRAF-MEKCraneofaringioma papilarCraneofaringioma adamantinomatosoMutación BRAF V600EWnt/beta-cateninaTratamiento dirigidoBRAF-MEK inhibitorsPapillary craniopharyngiomaAdamantinomatous craniopharyngiomaBRAF V600E mutationWnt/beta-cateninTargeted treatment61Background: Craniopharyngiomas, rare primary brain tumors of the pituitary-hypothalamic axis, frequently result in sicubstantial morbidity, including compromised quality of life, vision impairment, hypothalamic and endocrine dysfunction, and neuroendocrine disturbances. Of particular importance is the development of hypothalamic obesity, which affects up to 25% of patients at diagnosis and increases to 50% after treatment. Genotyping has revealed that over 90% of papillary craniopharyngiomas (PCP) harbor BRAF V600E mutations. Recent studies have demonstrated a significant reduction in tumor size with the use of BRAF-MEK inhibitors in PCP. Methods: We conducted a systematic review of recent literature on pretreatment or neo-adjuvant medical therapies, analyzing their effectiveness, safety, and sequelae following surgical treatment with this new approach. Results: At the time of this review, 15 studies involving more than 50 patients have been published, with a response rate of up to 90%. Conclusion: Based on this evidence, we propose a new treatment paradigm aimed at improving outcomes by maximizing relief from compressive symptoms while minimizing hypothalamic dysfunction.info:eu-repo/semantics/publishedVersionSpringer Nature2025info:eu-repo/semantics/article9http://hdl.handle.net/20.500.12328/5061https://dx.doi.org/10.1007/s12020-025-04216-9reponame:Recercat. Dipósit de la Recerca de Catalunyainstname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)InglésEndocrine89This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, which permits any non-commercial use, sharing, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if you modified the licensed material. You do not have permission under this licence to share adapted material derived from this article or parts of it. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by-nc-nd/4.0/.http://creativecommons.org/licenses/by-nc-nd/4.0/info:eu-repo/semantics/openAccessoai:recercat.cat:20.500.12328/50612026-05-29T05:05:01Z
dc.title.none.fl_str_mv Modern treatment of craniopharyngioma to improve outcomes: evidence of a change of paradigm
title Modern treatment of craniopharyngioma to improve outcomes: evidence of a change of paradigm
spellingShingle Modern treatment of craniopharyngioma to improve outcomes: evidence of a change of paradigm
González-Gallego, Carlos
Inhibidors de BRAF-MEK
Craniofaringioma papil·lar
Craniofaringioma adamantinomatós
Mutació BRAF V600E
Wnt/beta-catenina
Tractament dirigit
Inhibidores de BRAF-MEK
Craneofaringioma papilar
Craneofaringioma adamantinomatoso
Mutación BRAF V600E
Wnt/beta-catenina
Tratamiento dirigido
BRAF-MEK inhibitors
Papillary craniopharyngioma
Adamantinomatous craniopharyngioma
BRAF V600E mutation
Wnt/beta-catenin
Targeted treatment
61
title_short Modern treatment of craniopharyngioma to improve outcomes: evidence of a change of paradigm
title_full Modern treatment of craniopharyngioma to improve outcomes: evidence of a change of paradigm
title_fullStr Modern treatment of craniopharyngioma to improve outcomes: evidence of a change of paradigm
title_full_unstemmed Modern treatment of craniopharyngioma to improve outcomes: evidence of a change of paradigm
title_sort Modern treatment of craniopharyngioma to improve outcomes: evidence of a change of paradigm
dc.creator.none.fl_str_mv González-Gallego, Carlos
Molina, Pedro
Hostalot, Cristina
Oliva, Anna
Blanco, Alberto
Puyalto de Pablo, Paloma
Comas, Silvia
Carrato, Cristina
Valassi, Elena
Puig-Domingo, Manel
author González-Gallego, Carlos
author_facet González-Gallego, Carlos
Molina, Pedro
Hostalot, Cristina
Oliva, Anna
Blanco, Alberto
Puyalto de Pablo, Paloma
Comas, Silvia
Carrato, Cristina
Valassi, Elena
Puig-Domingo, Manel
author_role author
author2 Molina, Pedro
Hostalot, Cristina
Oliva, Anna
Blanco, Alberto
Puyalto de Pablo, Paloma
Comas, Silvia
Carrato, Cristina
Valassi, Elena
Puig-Domingo, Manel
author2_role author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Inhibidors de BRAF-MEK
Craniofaringioma papil·lar
Craniofaringioma adamantinomatós
Mutació BRAF V600E
Wnt/beta-catenina
Tractament dirigit
Inhibidores de BRAF-MEK
Craneofaringioma papilar
Craneofaringioma adamantinomatoso
Mutación BRAF V600E
Wnt/beta-catenina
Tratamiento dirigido
BRAF-MEK inhibitors
Papillary craniopharyngioma
Adamantinomatous craniopharyngioma
BRAF V600E mutation
Wnt/beta-catenin
Targeted treatment
61
topic Inhibidors de BRAF-MEK
Craniofaringioma papil·lar
Craniofaringioma adamantinomatós
Mutació BRAF V600E
Wnt/beta-catenina
Tractament dirigit
Inhibidores de BRAF-MEK
Craneofaringioma papilar
Craneofaringioma adamantinomatoso
Mutación BRAF V600E
Wnt/beta-catenina
Tratamiento dirigido
BRAF-MEK inhibitors
Papillary craniopharyngioma
Adamantinomatous craniopharyngioma
BRAF V600E mutation
Wnt/beta-catenin
Targeted treatment
61
description Background: Craniopharyngiomas, rare primary brain tumors of the pituitary-hypothalamic axis, frequently result in sicubstantial morbidity, including compromised quality of life, vision impairment, hypothalamic and endocrine dysfunction, and neuroendocrine disturbances. Of particular importance is the development of hypothalamic obesity, which affects up to 25% of patients at diagnosis and increases to 50% after treatment. Genotyping has revealed that over 90% of papillary craniopharyngiomas (PCP) harbor BRAF V600E mutations. Recent studies have demonstrated a significant reduction in tumor size with the use of BRAF-MEK inhibitors in PCP. Methods: We conducted a systematic review of recent literature on pretreatment or neo-adjuvant medical therapies, analyzing their effectiveness, safety, and sequelae following surgical treatment with this new approach. Results: At the time of this review, 15 studies involving more than 50 patients have been published, with a response rate of up to 90%. Conclusion: Based on this evidence, we propose a new treatment paradigm aimed at improving outcomes by maximizing relief from compressive symptoms while minimizing hypothalamic dysfunction.
publishDate 2025
dc.date.none.fl_str_mv 2025
dc.type.none.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv http://hdl.handle.net/20.500.12328/5061
https://dx.doi.org/10.1007/s12020-025-04216-9
url http://hdl.handle.net/20.500.12328/5061
https://dx.doi.org/10.1007/s12020-025-04216-9
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Endocrine
89
dc.rights.none.fl_str_mv http://creativecommons.org/licenses/by-nc-nd/4.0/
info:eu-repo/semantics/openAccess
rights_invalid_str_mv http://creativecommons.org/licenses/by-nc-nd/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv 9
dc.publisher.none.fl_str_mv Springer Nature
publisher.none.fl_str_mv Springer Nature
dc.source.none.fl_str_mv reponame:Recercat. Dipósit de la Recerca de Catalunya
instname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
instname_str Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
reponame_str Recercat. Dipósit de la Recerca de Catalunya
collection Recercat. Dipósit de la Recerca de Catalunya
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15,81155

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