{"id":145,"date":"2022-09-28T17:15:16","date_gmt":"2022-09-28T17:15:16","guid":{"rendered":"https:\/\/live-rawf.pantheonsite.io\/2022\/09\/28\/2022-9-28-2022-research-progress-on-craniopharyngioma\/"},"modified":"2025-12-23T02:58:47","modified_gmt":"2025-12-23T02:58:47","slug":"2022-9-28-2022-progres-de-la-recherche-sur-le-craniopharyngiome","status":"publish","type":"post","link":"https:\/\/www.rawoodfoundation.org\/fr\/2022\/09\/28\/2022-9-28-2022-research-progress-on-craniopharyngioma\/","title":{"rendered":"2022 Progr\u00e8s de la recherche sur le craniopharyngiome"},"content":{"rendered":"
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par Nathalie Kayadjanian, Ph.D., conseill\u00e8re scientifique du RAWF<\/em><\/p>\n<\/div>\n

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Cr\u00e9dit photo : National Cancer Institute<\/p>\n<\/div>\n<\/figcaption><\/figure>\n<\/div>\n

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Bien que la chirurgie et la radioth\u00e9rapie repr\u00e9sentent les th\u00e9rapies de premi\u00e8re ligne recommand\u00e9es pour la PC, elles sont en partie responsables des comorbidit\u00e9s associ\u00e9es \u00e0 la PC, notamment les alt\u00e9rations m\u00e9taboliques primaires, telles que l'ob\u00e9sit\u00e9, les troubles neuro-endocriniens et les alt\u00e9rations psychosociales. Les progr\u00e8s r\u00e9cents dans la compr\u00e9hension des alt\u00e9rations g\u00e9nomiques et des voies mol\u00e9culaires impliqu\u00e9es dans la tumorigen\u00e8se de la PC ont contribu\u00e9 \u00e0 faire progresser les diagnostics et les tests de m\u00e9dicaments ciblant ces voies. Un exemple r\u00e9ussi est la r\u00e9duction significative de la tumeur mesur\u00e9e chez les patients atteints d'une PC \u00e0 l'\u00e2ge adulte trait\u00e9s avec des inhibiteurs de BRAF et de MEK, rapport\u00e9e r\u00e9cemment dans plusieurs \u00e9tudes cliniques et un essai clinique de phase II (Fasano et al., 2022). La d\u00e9tection des mutations BRAF dans le s\u00e9rum (Brastianos et al.2016) pourrait contribuer aux \u00e9valuations diagnostiques pr\u00e9op\u00e9ratoires dans un avenir proche. Dans l'ensemble, ces r\u00e9sultats ouvrent la voie \u00e0 un diagnostic non op\u00e9ratoire et \u00e0 une strat\u00e9gie de traitement n\u00e9oadjuvant \u00e0 vis\u00e9e mol\u00e9culaire pour les patients atteints de CP.<\/p>\n

D\u00e9ficit en hormone de croissance (GH)<\/a> affecte le sch\u00e9ma de croissance chez les enfants atteints de PC (75%). Chez les adultes atteints de PC, le d\u00e9ficit en GH se caract\u00e9rise par une diminution de la masse musculaire, une augmentation de la graisse corporelle, une r\u00e9duction de l'\u00e9nergie et du bien-\u00eatre. La th\u00e9rapie de remplacement de la GH est la seule th\u00e9rapie efficace. Outre son effet positif sur la croissance des enfants, le remplacement de la GH a des effets b\u00e9n\u00e9fiques sur la composition corporelle, la sant\u00e9 osseuse, les r\u00e9sultats m\u00e9taboliques et la qualit\u00e9 de vie. Bien que le traitement par GH ait suscit\u00e9 des inqui\u00e9tudes en raison de ses propri\u00e9t\u00e9s pro-prolif\u00e9ratives, angiog\u00e9niques et anti-apoptotiques connues, de nombreuses \u00e9tudes ont d\u00e9montr\u00e9 que le traitement substitutif par GH n'augmente pas le risque de r\u00e9cidive tumorale chez les patients atteints de PC. Cette ann\u00e9e, une d\u00e9claration de consensus en faveur de la th\u00e9rapie par la GH dans la PC a \u00e9t\u00e9 publi\u00e9e (Boguszewski et al., 2022).<\/p>\n

Deux types de CP, l'adamantinomateux (ACP) et le papillaire (PCP), ont \u00e9t\u00e9 pr\u00e9c\u00e9demment d\u00e9crits sur la base de leurs caract\u00e9ristiques histologiques. Gr\u00e2ce aux progr\u00e8s mol\u00e9culaires et cliniques, l'OMS a modifi\u00e9 la taxonomie des tumeurs c\u00e9r\u00e9brales du SNC (5e \u00e9dition), de sorte que l'ACP et la PCP sont d\u00e9sormais reconnues comme des types distincts de tumeurs de la PC, compte tenu de leurs diff\u00e9rents profils clinicod\u00e9mographiques, radiologiques, histopathologiques et d'alt\u00e9ration g\u00e9n\u00e9tique. Bien qu'elle puisse toucher les adultes, l'ACP est surtout une tumeur p\u00e9diatrique et repr\u00e9sente la tumeur hypophysaire la plus fr\u00e9quente chez les enfants. Traitement du craniopharyngiome chez l'enfant<\/a> se concentre sur l'\u00e9quilibre entre l'ablation efficace de la tumeur et la pr\u00e9servation de la fonction hormonale et de la qualit\u00e9 de vie. En revanche, la PCP touche principalement les adultes.<\/p>\n

La recherche sur la PC manque de donn\u00e9es factuelles sur les points de vue des patients et des soignants concernant les sympt\u00f4mes importants et les priorit\u00e9s de traitement. Une enqu\u00eate aupr\u00e8s des patients et des soignants a r\u00e9cemment \u00e9t\u00e9 publi\u00e9e afin de mieux comprendre les approches adopt\u00e9es par les familles en mati\u00e8re de gestion du poids et l'impact sur la fonction sociale des personnes atteintes de PC et d'ob\u00e9sit\u00e9 (Craven et al., 2022). Il est int\u00e9ressant de noter que les r\u00e9sultats soulignent la possibilit\u00e9 d'am\u00e9liorer le fonctionnement social en tant que b\u00e9n\u00e9fice potentiel suppl\u00e9mentaire de traitements am\u00e9lior\u00e9s de l'ob\u00e9sit\u00e9 hypothalamique.<\/p>\n

Parall\u00e8lement \u00e0 l'am\u00e9lioration des r\u00e9sultats chirurgicaux, la norme pour une chirurgie r\u00e9ussie s'est \u00e9largie pour se concentrer sur la qualit\u00e9 de vie d\u00e9clar\u00e9e par le patient (QoL). Une \u00e9tude r\u00e9cente a montr\u00e9 que la qualit\u00e9 de vie des patients atteints de PC \u00e9tait inf\u00e9rieure \u00e0 celle de la population g\u00e9n\u00e9rale (Castle-Kirszbaum et al., 2022). L'atteinte hypothalamique \u00e9tait syst\u00e9matiquement le facteur pr\u00e9dictif le plus important de la qualit\u00e9 de vie. L'endocrinopathie contribuait \u00e0 la morbidit\u00e9 mais pouvait \u00eatre am\u00e9lior\u00e9e par un traitement hormonal substitutif. La dysr\u00e9gulation sociale et \u00e9motionnelle et une mauvaise m\u00e9moire sont des plaintes courantes apr\u00e8s la chirurgie. Cette \u00e9tude a mis en lumi\u00e8re des comorbidit\u00e9s peu \u00e9tudi\u00e9es qui affectent de mani\u00e8re significative la qualit\u00e9 de vie des patients atteints de PC.<\/p>\n

Les troubles du sommeil repr\u00e9sentent un probl\u00e8me important chez les patients atteints de PC. Ils restent cependant largement sous-diagnostiqu\u00e9s et non trait\u00e9s dans la pratique clinique et il n'existe pas de lignes directrices pour le diagnostic et la prise en charge des troubles du sommeil chez ces patients. Romigi et coll. (2022) ont publi\u00e9 un article r\u00e9cent sur les connaissances actuelles de la pathogen\u00e8se et de la pr\u00e9valence des troubles du sommeil chez les patients atteints de PC et proposent des algorithmes pratiques pour leur \u00e9valuation et leur prise en charge dans la pratique clinique.<\/p>\n

Liens connexes :<\/strong>
\nEssais cliniques en cours sur le craniopharyngiome<\/a>
\nEnqu\u00eate sur les r\u00e9sultats pertinents pour les patients atteints de craniopharyngiome p\u00e9diatrique : L'ob\u00e9sit\u00e9 hypothalamique en point de mire<\/a>
\nRessources pour les personnes nouvellement diagnostiqu\u00e9es<\/a><\/p>\n

R\u00e9f\u00e9rences<\/strong><\/p>\n

Boguszewski MCS et al, (2022). S\u00e9curit\u00e9 du remplacement de l'hormone de croissance chez les survivants du cancer et des tumeurs intracr\u00e2niennes et hypophysaires : une d\u00e9claration de consensus. Eur J Endocrinol. 2022 Apr 21;186(6):P35-P52.<\/p>\n

Castle-Kirszbaum M, et al, (2022). Quality of Life in Craniopharyngioma : A Systematic Review. World Neurosurg. Aug;164:424-435.e2. doi : 10.1016\/j.wneu.2022.05.038.<\/p>\n

Craven M, et al (2022) A Survey of Patient-Relevant Outcomes in Pediatric Craniopharyngioma : Focus on Hypothalamic Obesity. Front. Endocrinol. 13:876770.<\/p>\n

Fasano M et al, (2022) Dramatic Therapeutic Response to Dabrafenib Plus Trametinib in BRAFV600E Mutated Papillary Craniopharyngiomas : A Case Report and Literature Review. Front. Med. 8:652005.<\/p>\n

Romigi A. et al, (2022), Sleep Disorders in Patients with Craniopharyngioma : A Physiopathological and Practical Update. Front Neurol. 2022 Feb 9;12:817257. doi : 10.3389\/fneur.2021.817257.<\/p>\n<\/div>","protected":false},"excerpt":{"rendered":"

par Nathalie Kayadjanian, Ph.D., conseill\u00e8re scientifique de RAWF Image : National Cancer Institute Alors que la chirurgie et la ... Lire plus<\/a><\/p>","protected":false},"author":6,"featured_media":146,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"footnotes":""},"categories":[35,20,1],"tags":[],"class_list":["post-145","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-craniopharyngioma","category-latest-research","category-uncategorized","generate-columns","tablet-grid-50","mobile-grid-100","grid-parent","grid-33","no-featured-image-padding"],"acf":[],"yoast_head":"\n2022 Research Progress on Craniopharyngioma - Raymond A. 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