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...
| Autores: | ; ; ; ; ; ; ; ; ; |
|---|---|
| Tipo de documento: | 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 |
| Nivel de acceso: | 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 |
| _version_ | 1878740709549277184 |
|---|---|
| author | González-Gallego, Carlos |
| 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 |
| author_browse | Blanco, Alberto Carrato, Cristina Comas, Silvia González-Gallego, Carlos Hostalot, Cristina Molina, Pedro Oliva, Anna Puig-Domingo, Manel Puyalto de Pablo, Paloma Valassi, Elena |
| 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 |
| collection | Recercat. Dipósit de la Recerca de Catalunya |
| 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. |
| eu_rights_str_mv | openAccess |
| format | article |
| id | ES_d7fed2d70220d28242529d5e611ffbae |
| institution | 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) |
| network_acronym_str | ES |
| network_name_str | España |
| oai_identifier_str | oai:recercat.cat:20.500.12328/5061 |
| publishDate | 2025 |
| publishDateSort | 2025 |
| publisher | Springer Nature |
| publisherStr | Springer Nature |
| reponame_str | Recercat. Dipósit de la Recerca de Catalunya |
| spelling | Modern treatment of craniopharyngioma to improve outcomes: evidence of a change of paradigm González-Gallego, Carlos Molina, Pedro Hostalot, Cristina Oliva, Anna Blanco, Alberto Puyalto de Pablo, Paloma Comas, Silvia Carrato, Cristina Valassi, Elena Puig-Domingo, Manel 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 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. info:eu-repo/semantics/publishedVersion Springer Nature http://hdl.handle.net/20.500.12328/5061 https://dx.doi.org/10.1007/s12020-025-04216-9 |
| 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 Tratamiento dirigido BRAF-MEK inhibitors Papillary craniopharyngioma Adamantinomatous craniopharyngioma BRAF V600E mutation Wnt/beta-catenin Targeted treatment 61 |
| title | 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_short | 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 |
| 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 Tratamiento dirigido BRAF-MEK inhibitors Papillary craniopharyngioma Adamantinomatous craniopharyngioma BRAF V600E mutation Wnt/beta-catenin Targeted treatment 61 |
| topic_facet | 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 |
| url | http://hdl.handle.net/20.500.12328/5061 https://dx.doi.org/10.1007/s12020-025-04216-9 |