{"id":2007,"date":"2025-12-11T15:05:48","date_gmt":"2025-12-11T12:05:48","guid":{"rendered":"https:\/\/wiki.dev.voka.io\/diseases\/uncategorized\/tumeurs-des-plexus-choroides\/"},"modified":"2025-12-11T15:05:51","modified_gmt":"2025-12-11T12:05:51","slug":"tumeurs-des-plexus-choroides","status":"publish","type":"diseases_post","link":"https:\/\/wiki.dev.voka.io\/fr\/maladies\/oncologiques\/tumeurs-des-plexus-choroides\/","title":{"rendered":"Tumeurs des plexus choro\u00efdes : papillome et carcinome \u2013 \u00e9tiologie, classification et traitement"},"content":{"rendered":"<p><?xml encoding=\"UTF-8\" ?><\/p>\n<p>Les plexus choro\u00efdes produisent le liquide c\u00e9phalo-rachidien (LCR) et sont situ\u00e9s dans les ventricules c\u00e9r\u00e9braux. La localisation tumorale d\u00e9pend de l\u2019\u00e2ge : chez l\u2019enfant, les ventricules lat\u00e9raux sont le plus souvent atteints ; chez l\u2019adulte, le IVe ventricule. <\/p>\n<p><strong>Papillome des plexus choro\u00efdes <\/strong> (PPC) \u2014  tumeur b\u00e9nigne de l\u2019\u00e9pith\u00e9lium des plexus choro\u00efdes du cerveau, class\u00e9e grade 1 OMS. Repr\u00e9sente la forme la plus fr\u00e9quente des tumeurs des plexus choro\u00efdes.<\/p>\n<figure class=\"wp-block-image size-large\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/articles\/en\/oncology\/choroid-plexus-tumors\/choroid-plexus-papilloma.webp\" alt=\"Papillome des plexus choro\u00efdes dans le IVe ventricule\"><figcaption class=\"wp-element-caption\">Papillome des plexus choro\u00efdes dans le IVe ventricule \u2013 <a href=\"https:\/\/catalog.voka.io\/en\/models\/cec5f80b-babf-4f02-ab97-355cf37c21ad\/95bcd6fa-2d66-476f-88bf-66e25519f4c9\/1b81e9e6-db72-4320-ace3-54319fac1292\/b3439e1b-466c-4922-9611-e6f42ca05078\" target=\"_blank\" rel=\"noreferrer noopener nofollow\">mod\u00e8le 3D<\/a><\/figcaption><\/figure>\n<p><strong>Carcinome des plexus choro\u00efdes <\/strong> (CPC) \u2014 tumeur rare hautement maligne (grade 3 OMS), issue de l\u2019\u00e9pith\u00e9lium des plexus choro\u00efdes ventriculaires. Diagnostiqu\u00e9e principalement chez l\u2019enfant de moins de 5 ans, caract\u00e9ris\u00e9e par une \u00e9volution agressive, une tendance \u00e0 l\u2019invasion, une diss\u00e9mination dans le syst\u00e8me nerveux central et un pronostic extr\u00eamement d\u00e9favorable.<\/p>\n<h2 class=\"wp-block-heading\" id=\"etiologie-et-epidemiologie-des-tumeurs\"><strong>\u00c9tiologie et \u00e9pid\u00e9miologie des tumeurs <\/strong><strong><\/strong><\/h2>\n<h3 class=\"wp-block-heading\" id=\"etiologie-et-facteurs-genetiques\"><strong>\u00c9tiologie et facteurs g\u00e9n\u00e9tiques<\/strong><\/h3>\n<p>Les causes exactes des PPC restent inconnues. Les PPS ne pr\u00e9sentent pas de mutations pilotes, mais certains cas sont associ\u00e9s \u00e0 des syndromes g\u00e9n\u00e9tiques rares, par exemple le syndrome d\u2019Aicardi.<\/p>\n<ul class=\"wp-block-list\">\n<li><strong>Les formes atypiques (aPCC) et malignes (CPC):<\/strong> peuvent survenir sur fond de mutations TP53, notamment chez les enfants atteints du syndrome de Li-Fraumeni.<\/li>\n<li>Jusqu\u2019\u00e0 60 % des patients atteints de CPC pr\u00e9sentent une mutation somatique TP53 ; dans 40 % des cas, la tumeur est associ\u00e9e au syndrome de Li-Fraumeni li\u00e9 \u00e0 une mutation germinale <em>TP53<\/em>.<\/li>\n<li>Dans certains cas, d\u2019autres alt\u00e9rations des g\u00e8nes de r\u00e9gulation du cycle cellulaire sont d\u00e9tect\u00e9es (par exemple <em>PTEN, CDKN2A<\/em>).<\/li>\n<\/ul>\n<h3 class=\"wp-block-heading\" id=\"epidemiologie\"><strong>\u00c9pid\u00e9miologie<\/strong><\/h3>\n<p>Les caract\u00e9ristiques \u00e9pid\u00e9miologiques des tumeurs des plexus choro\u00efdes sont conditionn\u00e9es par leur faible incidence et leur forte d\u00e9pendance \u00e0 l\u2019\u00e2ge du patient.<\/p>\n<ul class=\"wp-block-list\">\n<li><strong>Fr\u00e9quence : <\/strong> Les tumeurs des plexus choro\u00efdes sont rares (moins de 1 % de l\u2019ensemble des tumeurs intracr\u00e2niennes). Chez l\u2019enfant de moins de 1 an, elles repr\u00e9sentent 10 \u00e0 15 % des n\u00e9oplasmes intracr\u00e2niens.<\/li>\n<li><strong>Pic d\u2019\u00e2ge : <\/strong>  les trois premi\u00e8res ann\u00e9es de vie.<\/li>\n<li><strong>Localisation : <\/strong>  chez l\u2019enfant, pr\u00e9dominance dans les ventricules lat\u00e9raux ; chez l\u2019adulte, dans le IVe ventricule. R\u00e9partition par sexe approximativement \u00e9gale.<\/li>\n<\/ul>\n<h2 class=\"wp-block-heading\" id=\"manifestations-cliniques-et-symptomes-des-tumeurs-des-plexus-choroides\"><strong>Manifestations cliniques et sympt\u00f4mes des tumeurs des plexus choro\u00efdes<\/strong><\/h2>\n<p>Le sympt\u00f4me principal est l\u2019hypertension intracr\u00e2nienne secondaire \u00e0 une hydroc\u00e9phalie (hyperproduction de LCR).  Se manifeste par des c\u00e9phal\u00e9es ; chez le nourrisson, par une augmentation des dimensions de la t\u00eate.<\/p>\n<ul class=\"wp-block-list\">\n<li><strong>Autres signes : <\/strong> naus\u00e9es, vomissements, crises convulsives, h\u00e9mipar\u00e9sie, l\u00e9thargie, troubles visuels (selon la localisation).<\/li>\n<li>Dans de rares cas, h\u00e9morragie intracr\u00e2nienne possible.<\/li>\n<\/ul>\n<figure class=\"wp-block-video\" id=\"animation-3d-papillome-des-plexus-choroides\"><video controls><source data-src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/articles\/en\/oncology\/choroid-plexus-tumors\/choroid-plexus-papilloma.webm\" type=\"video\/webm\"><\/source><\/video><figcaption class=\"wp-element-caption\">Animation 3D \u2013 papillome des plexus choro\u00efdes<\/figcaption><\/figure>\n<h2 class=\"wp-block-heading\" id=\"classification-des-tumeurs-des-plexus-choroides\"><strong>Classification des tumeurs des plexus choro\u00efdes<\/strong><\/h2>\n<p>La classification actuelle (OMS, 5e \u00e9dition, 2021) repose sur les caract\u00e9ristiques morphologiques, immunohistochimiques et mol\u00e9culaires.<\/p>\n<h3 class=\"wp-block-heading\" id=\"grade-oms-grade-de-malignite\"><strong>Grade OMS (grade de malignit\u00e9)<\/strong><\/h3>\n<figure class=\"wp-block-table table-to-cards\">\n<table class=\"has-fixed-layout\">\n<thead>\n<tr>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong>Grade OMS<\/strong><\/th>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong>Nom<\/strong><\/th>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong>Caract\u00e9ristiques<\/strong><\/th>\n<\/tr>\n<\/thead>\n<tbody>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>Grade 1<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>Papillome des plexus choro\u00efdes (PPC)<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Forme b\u00e9nigne<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>Grade 2<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>Papillome atypique des plexus choro\u00efdes (aPPC)<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Activit\u00e9 mitotique augment\u00e9e ; risque de r\u00e9cidive plus \u00e9lev\u00e9<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>Grade 3<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>Carcinome des plexus choro\u00efdes (CPC)<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Maligne, invasive<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/figure>\n<h3 class=\"wp-block-heading\" id=\"classification-moleculaire\"><strong>Classification mol\u00e9culaire <\/strong><\/h3>\n<p>Les \u00e9tudes r\u00e9centes distinguent trois sous-types mol\u00e9culaires d\u00e9finis par le profil de m\u00e9thylation et l\u2019expression g\u00e9nique :<\/p>\n<ol class=\"wp-block-list\">\n<li>P\u00e9diatrique supratentoriel low-risk (<strong>\u0430PPC<\/strong>)<\/li>\n<li>P\u00e9diatrique supratentoriel high-risk (<strong>CPC<\/strong>)<\/li>\n<li>Adulte infratentoriel low-risk (<strong>\u0430PPC<\/strong>)<\/li>\n<\/ol>\n<p>Ainsi, en pratique clinique, la morphologie, l\u2019activit\u00e9 prolif\u00e9rative, les marqueurs mol\u00e9culaires et le statut TP53 <strong>,<\/strong> sont pris en compte pour d\u00e9terminer le pronostic et l\u2019intensit\u00e9 th\u00e9rapeutique.<\/p>\n<h2 class=\"wp-block-heading\" id=\"diagnostic-et-diagnostic-differentiel\"><strong>Diagnostic et diagnostic diff\u00e9rentiel<\/strong><\/h2>\n<h3 class=\"wp-block-heading\" id=\"methodes-diagnostiques\"><strong>M\u00e9thodes diagnostiques<\/strong><\/h3>\n<p>M\u00e9thode principale : IRM. En cas d\u2019impossibilit\u00e9 : TDM ou \u00e9chographie (chez le tr\u00e8s jeune enfant par la fontanelle).<\/p>\n<ul class=\"wp-block-list\">\n<li><strong>IRM et TDM :<\/strong> la tumeur des plexus choro\u00efdes est g\u00e9n\u00e9ralement hyperintense en T2, souvent calcifi\u00e9e au TDM, accumule intens\u00e9ment le contraste.<\/li>\n<\/ul>\n<p><strong>Pour le CPC : <\/strong> h\u00e9t\u00e9rog\u00e9n\u00e9it\u00e9 structurale, zones de n\u00e9crose, invasion, rehaussement marqu\u00e9, \u0153d\u00e8me vasog\u00e9nique p\u00e9ril\u00e9sionnel.<\/p>\n<ul class=\"wp-block-list\">\n<li><strong>Diagnostic mol\u00e9culaire : <\/strong>d\u00e9pistage du syndrome de Li-Fraumeni (mutation germinale TP53) obligatoire pour tout CPC.<\/li>\n<li><strong>Ponction lombaire :  <\/strong>r\u00e9alis\u00e9e pour rechercher des cellules tumorales dans le LCR (CPC).<\/li>\n<\/ul>\n<h3 class=\"wp-block-heading\" id=\"diagnostic-differentiel\"><strong>Diagnostic diff\u00e9rentiel<\/strong><\/h3>\n<figure class=\"wp-block-table table-to-cards\">\n<table class=\"has-fixed-layout\">\n<thead>\n<tr>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong>Type de tumeur<\/strong><\/th>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong>Chez l\u2019enfant (exemples)<\/strong><\/th>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong>Chez l\u2019adulte (exemples)<\/strong><\/th>\n<\/tr>\n<\/thead>\n<tbody>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>PPC\/aPPC<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">\u00c9pendymome, m\u00e9dulloblastome, astrocytome pilocytique<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">M\u00e9ningiome papillaire, ad\u00e9nocarcinome m\u00e9tastatique, tumeur fibreuse solitaire<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>CPC<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Tumeur t\u00e9rato\u00efde\/rhabdo\u00efde atypique (ATRT), tumeur embryonnaire (ETMR), \u00e9pendymome, m\u00e9tastases (rares)<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">M\u00e9tastases, choriocarcinome, m\u00e9ningiome papillaire haut grade<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/figure>\n<p>Ainsi, chez l\u2019enfant, le diagnostic diff\u00e9rentiel porte principalement sur les \u00e9pendymomes et les tumeurs embryonnaires ; chez l\u2019adulte, sur les m\u00e9ningiomes et les carcinomes m\u00e9tastatiques.<\/p>\n<div class=\"social-banner-block\">\n<div class=\"social-banner-content\">\n<p class=\"h5-title text-black\">Retrouvez d\u2019autres contenus scientifiquement exacts sur nos m\u00e9dias sociaux<\/p>\n<p><span class=\"social-banner-text text-grey\">Abonnez-vous et ne manquez pas les derni\u00e8res ressources<\/span><\/p>\n<div class=\"social-links-wrapper\"><a class=\"social-icon-link\" href=\"https:\/\/www.facebook.com\/VOKA3DAnatomyAndPathology\/\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/facebook.svg\" alt=\"social link\"><\/a><a class=\"social-icon-link\" href=\"https:\/\/www.instagram.com\/voka.io\/\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/insta.svg\" alt=\"social link\"><\/a><a class=\"social-icon-link\" href=\"https:\/\/www.linkedin.com\/company\/voka-io\/posts\/?feedView=all\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/linkedin.svg\" alt=\"social link\"><\/a><a class=\"social-icon-link\" href=\"https:\/\/www.youtube.com\/@vokaio\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/youtube.svg\" alt=\"social link\"><\/a><a class=\"social-icon-link\" href=\"https:\/\/www.pinterest.com\/voka3danatomyandpathology\/\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/pinterest.svg\" alt=\"social link\"><\/a><a class=\"social-icon-link\" href=\"https:\/\/www.tiktok.com\/@voka.io\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/tiktok.svg\" alt=\"social link\"><\/a><a class=\"social-icon-link\" href=\"https:\/\/discord.gg\/7ejUpq8DRR\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/discord.svg\" alt=\"social link\"><\/a><\/div>\n<\/div>\n<p><img decoding=\"async\" class=\"social-banner-image\" loading=\"lazy\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/social-media-banner-mobile-image.webp\" alt=\"Banner background\"><\/div>\n<h2 class=\"wp-block-heading\" id=\"methodes-de-traitement-et-strategies-pronostiques-des-tumeurs-des-plexus-choroides\"><strong>M\u00e9thodes de traitement et strat\u00e9gies pronostiques des tumeurs des plexus choro\u00efdes<\/strong><\/h2>\n<p>Le traitement est multimodal et d\u00e9bute par la chirurgie, qui conditionne la strat\u00e9gie ult\u00e9rieure et le pronostic.<\/p>\n<h3 class=\"wp-block-heading\" id=\"1-traitement-chirurgical\">1. <strong>Traitement chirurgical<\/strong><\/h3>\n<ul class=\"wp-block-list\">\n<li><strong>Standard-or : <\/strong><strong>r\u00e9section totale macroscopique (gross total resection)<\/strong>. Les techniques endoscopiques sont souvent utilis\u00e9es.<\/li>\n<li><strong>Pronostic des PPC : <\/strong> m\u00eame une r\u00e9section subtotale dans les papillomes classiques des plexus choro\u00efdes assure fr\u00e9quemment un pronostic favorable.<\/li>\n<li><strong>Difficult\u00e9s des CPC : <\/strong> l\u2019ex\u00e9r\u00e8se compl\u00e8te est souvent entrav\u00e9e par l\u2019invasion tumorale et les saignements perop\u00e9ratoires.<\/li>\n<li><strong>Postop\u00e9ratoire :<\/strong> une d\u00e9rivation ventriculaire peut s\u2019av\u00e9rer n\u00e9cessaire en raison de dysfonction du LCR.<\/li>\n<\/ul>\n<h3 class=\"wp-block-heading\" id=\"2-radiotherapie\">2. <strong>Radioth\u00e9rapie<br \/>\n<\/strong><\/h3>\n<ul class=\"wp-block-list\">\n<li><strong>PPC :<\/strong> g\u00e9n\u00e9ralement non requise apr\u00e8s r\u00e9section radicale.<\/li>\n<li><strong>Indications : <\/strong> utilis\u00e9e en cas de r\u00e9cidives r\u00e9p\u00e9t\u00e9es, d\u2019impossibilit\u00e9 de r\u00e9section ad\u00e9quate ou pour les formes atypiques.<\/li>\n<li><strong>CPC : <\/strong> chez les patients de plus de 3 ans, irradiation craniospinale apr\u00e8s chimioth\u00e9rapie.<\/li>\n<li><strong>Limitations : <\/strong> \u00e9vit\u00e9e en premi\u00e8re intention chez l\u2019enfant en raison du risque de troubles cognitifs. Chez les enfants < de 3 ans et moins ainsi que chez les patients porteurs du syndrome de Li-Fraumeni, son utilisation est extr\u00eamement pond\u00e9r\u00e9e en raison du risque de tumeurs secondaires.<\/li>\n<\/ul>\n<h3 class=\"wp-block-heading\" id=\"3-chimiotherapie\">3. <strong>Chimioth\u00e9rapie<br \/>\n<\/strong><\/h3>\n<ul class=\"wp-block-list\">\n<li><strong>Indiqu\u00e9e <\/strong> chez les patients \u00e0 haut risque (CPC, aPPC avec r\u00e9sidu tumoral, m\u00e9tastases).<\/li>\n<li><strong>Sch\u00e9mas principaux : <\/strong> carboplatine\/\u00e9toposide\/vincristine (CarbEV) ; cyclophosphamide\/\u00e9toposide\/vincristine (CycEV). CarbEV sup\u00e9rieur en termes de survie dans les CPC.<\/li>\n<li>Dans certains cas, chimioth\u00e9rapie consolidante \u00e0 haute dose avec autogreffe de moelle osseuse chez les patients porteurs du syndrome de Li-Fraumeni.<\/li>\n<\/ul>\n<h3 class=\"wp-block-heading\" id=\"pronostic-et-facteurs-de-survie\"><strong>Pronostic et facteurs de survie<\/strong><\/h3>\n<ul class=\"wp-block-list\">\n<li><strong>Favorable : <\/strong> apr\u00e8s ex\u00e9r\u00e8se compl\u00e8te des aPPC survie \u00e0 10 ans sup\u00e9rieure \u00e0 90 %. Pour les aPPC, risque de r\u00e9cidive plus \u00e9lev\u00e9, traitement adjuvant souvent n\u00e9cessaire.<\/li>\n<li><strong>D\u00e9favorable :<\/strong> pronostic des CPC plus sombre (survie \u00e0 5 ans jusqu\u2019\u00e0 40 %) en pr\u00e9sence de mutation TP53. Pronostic aggrav\u00e9 par la pr\u00e9sence de syndromes g\u00e9n\u00e9tiques, de m\u00e9tastases, de r\u00e9section incompl\u00e8te et chez les patients atteints du syndrome de Li-Fraumeni.<\/li>\n<\/ul>\n<div>\n<h2 class=\"faq-title h2-article\" id=\"faq\">FAQ<\/h2>\n<div class=\"faq-section\">\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">1. En quoi le papillome des plexus choro\u00efdes diff\u00e8re-t-il du carcinome ?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Le papillome (PPC) est une tumeur b\u00e9nigne (grade 1 OMS) au pronostic favorable, alors que le carcinome (CPC) est une tumeur hautement maligne (grade 3 OMS) \u00e0 \u00e9volution agressive et pronostic d\u00e9favorable.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">2. Quel est le sympt\u00f4me principal des tumeurs des plexus choro\u00efdes ?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Le sympt\u00f4me principal est l\u2019hypertension intracr\u00e2nienne secondaire \u00e0 une hyperproduction de LCR (hydroc\u00e9phalie). Chez le nourrisson, elle se manifeste souvent par une augmentation des dimensions de la t\u00eate.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">3. Quel est le r\u00f4le de la mutation TP53 dans le diagnostic des CPC ? <\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">La mutation TP53 a une valeur pronostique majeure : elle est fr\u00e9quemment associ\u00e9e au syndrome de Li-Fraumeni et indique un pronostic plus d\u00e9favorable dans le carcinome des plexus choro\u00efdes.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">4. Quel traitement est consid\u00e9r\u00e9 comme le \u00ab standard-or \u00bb pour les PPC ?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Le standard-or du traitement du papillome des plexus choro\u00efdes (PPC) est la r\u00e9section chirurgicale totale macroscopique (gross total resection).<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">5. Pourquoi la radioth\u00e9rapie est-elle \u00e9vit\u00e9e dans le traitement des CPC chez le jeune enfant ?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">La radioth\u00e9rapie est \u00e9vit\u00e9e chez l\u2019enfant de moins de 3 ans en raison du risque \u00e9lev\u00e9 de troubles cognitifs s\u00e9v\u00e8res et de survenue de tumeurs secondaires, particuli\u00e8rement chez les patients porteurs du syndrome de Li-Fraumeni.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<\/div>\n<\/div>\n<div class=\"sources-list-block sources-list-hidden\" id=\"references\">\n<div class=\"sources-list-content\">\n<div class=\"sources-list-title\">\n<p class=\"small-text-bold text-black sources-list-title-text\">R\u00e9f\u00e9rences<\/p>\n<div class=\"sources-expand-button-wrapper-mobile\">\n<div class=\"sources-expand-button\"><svg width=\"32\" height=\"32\" viewbox=\"0 0 32 32\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\"><path d=\"M8 12L16 20L24 12\" stroke=\"#8C9AAB\" stroke-width=\"2\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><\/path><\/svg><\/div>\n<\/div>\n<\/div>\n<div class=\"sources-list-items\">\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">1.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Catalogue VOKA. [Ressource \u00e9lectronique]<\/cite><\/p>\n<p><span class=\"small-text-medium text-grey\">https:\/\/catalog.voka.io\/<\/span><\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">2.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Wolff JE, et al. Final results of the Choroid Plexus Tumor study CPT-SIOP-2000. [R\u00e9sultats finaux de l\u2019\u00e9tude CPT-SIOP-2000 sur les tumeurs des plexus choro\u00efdes.]. J Neurooncol 2022;156(3):599\u2013613. <\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">3.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Louis DN, et al. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. [Classification OMS 2021 des tumeurs du syst\u00e8me nerveux central : r\u00e9sum\u00e9.]. Neuro Oncol. 2021;23:1231\u20131251.<\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">4.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Thomas C, et al. Molecular genetics and diversity of choroid plexus tumors. [G\u00e9n\u00e9tique mol\u00e9culaire et diversit\u00e9 des tumeurs des plexus choro\u00efdes.]. Neuro-Oncol Adv 2024.<\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">5.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Andour H, et al. Atypical choroid plexus papilloma: Diagnosis and management. [Papillome atypique des plexus choro\u00efdes : diagnostic et prise en charge.]. SAGE Open Med. 2024; doi:10.1177\/2050313X241254000.<\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">6.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Yankelevich M, et al. Marrow-ablative consolidation chemotherapy and molecular targeted therapy for CPC. [Chimioth\u00e9rapie de consolidation my\u00e9loablatrice et th\u00e9rapie mol\u00e9culaire cibl\u00e9e pour le carcinome des plexus choro\u00efdes.] Neuro-Oncol Adv 2024;6(1):vdae109.<\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">7.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Cornelius A, et al. Molecular Guided Therapy Provides Sustained Clinical Remission in Pediatric CPC. [Th\u00e9rapie guid\u00e9e par les donn\u00e9es mol\u00e9culaires permet une r\u00e9mission clinique prolong\u00e9e dans le carcinome des plexus choro\u00efdes p\u00e9diatrique.] Frontiers Pharmacol. 2017;8:652.<\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">8.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Wolff JE, Sajedi M, Brant R. Choroid plexus tumours.[Tumeurs des plexus choro\u00efdes.]. Br J Cancer. 2002 87:1086\u201391.<\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">9.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Safaee M, et al. Choroid plexus papillomas: advances in molecular biology and surgical management. [Papillomes des plexus choro\u00efdes : avanc\u00e9es en biologie mol\u00e9culaire et prise en charge chirurgicale.] Neurosurg Focus. 2012;32(2):E6.<\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">10.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Akade E, et al. Diagnosis of choroid plexus papilloma. [Diagnostic du papillome des plexus choro\u00efdes.] Curr Probl Cancer. 2024; doi:10.1016\/j.cpt.2023.09.005.<\/cite><\/p>\n<\/div>\n<\/div>\n<\/div>\n<\/div>\n<div class=\"sources-expand-button-wrapper\">\n<div class=\"sources-expand-button\"><svg width=\"32\" height=\"32\" viewbox=\"0 0 32 32\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\"><path d=\"M8 12L16 20L24 12\" stroke=\"#8C9AAB\" stroke-width=\"2\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><\/path><\/svg><\/div>\n<\/div>\n<\/div>\n","protected":false},"excerpt":{"rendered":"<p>Les plexus choro\u00efdes produisent le liquide c\u00e9phalo-rachidien (LCR) et sont situ\u00e9s dans les ventricules c\u00e9r\u00e9braux. La localisation tumorale d\u00e9pend de l\u2019\u00e2ge : chez l\u2019enfant, les ventricules lat\u00e9raux sont le plus souvent atteints ; chez l\u2019adulte, le IVe ventricule. Papillome des plexus choro\u00efdes (PPC) \u2014 tumeur b\u00e9nigne de l\u2019\u00e9pith\u00e9lium des plexus choro\u00efdes du cerveau, class\u00e9e grade [&hellip;]<\/p>\n","protected":false},"author":11,"featured_media":0,"template":"","diseases_category":[350],"class_list":["post-2007","diseases_post","type-diseases_post","status-publish","hentry","diseases_category-oncologiques"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v25.0 (Yoast SEO v26.5) - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Tumeurs des plexus choro\u00efdes (TPC) : papillome et carcinome \u2013 diagnostic et traitement<\/title>\n<meta name=\"description\" content=\"Guide complet des tumeurs des plexus choro\u00efdes (PPC et CPC) : \u00e9tiologie, sympt\u00f4mes, classification OMS, diagnostic mol\u00e9culaire et m\u00e9thodes de traitement.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" 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