Modern treatment of craniopharyngioma to improve outcomes : evidence of a change of paradigm
González-Gallego, Carlos (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Molina, Pedro (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Hostalot, Cristina (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Oliva, Anna (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Blanco, Alberto (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Puyalto de Pablo, Paloma (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Comas, Silvia (Institut Català d'Oncologia)
Carrato, Cristina (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Valassi, Elena (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Puig Domingo, Manuel (Universitat Autònoma de Barcelona. Departament de Medicina)

Data:	2025
Resum:	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. 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. At the time of this review, 15 studies involving more than 50 patients have been published, with a response rate of up to 90%. Based on this evidence, we propose a new treatment paradigm aimed at improving outcomes by maximizing relief from compressive symptoms while minimizing hypothalamic dysfunction.
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, i la comunicació pública de l'obra, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original. No es permet la creació d'obres derivades.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Matèria:	BRAF-MEK inhibitors ; Papillary craniopharyngioma ; Adamantinomatous craniopharyngioma ; BRAF V600E mutation ; Wnt/beta-catenin ; Targeted treatment
Publicat a:	Endocrine, Vol. 89 Núm. 1 (May 2025) , p. 20-29, ISSN 1559-0100

DOI: 10.1007/s12020-025-04216-9
PMID: 40347305

10 p, 780.4 KB

El registre apareix a les col·leccions:
Articles > Articles de recerca
Articles > Articles publicats