{"id":21281,"date":"2024-08-07T10:14:05","date_gmt":"2024-08-07T10:14:05","guid":{"rendered":"https:\/\/site-euspringworks-live-1721707192-jq9cikhfcu2uwhr.local\/a-propos-des-tumeurs-desmoides\/"},"modified":"2025-06-16T13:41:55","modified_gmt":"2025-06-16T13:41:55","slug":"a-propos-des-tumeurs-desmoides","status":"publish","type":"page","link":"https:\/\/www.desmoidtumours.eu\/fr\/a-propos-des-tumeurs-desmoides\/","title":{"rendered":"\u00c0 propos des tumeurs desmo\u00efdes\u200b"},"content":{"rendered":"\t\t<div data-elementor-type=\"wp-page\" data-elementor-id=\"21281\" class=\"elementor elementor-21281 elementor-20245\" data-elementor-post-type=\"page\">\n\t\t\t\t<div class=\"elementor-element elementor-element-c902066 e-flex e-con-boxed e-con e-parent\" data-id=\"c902066\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t\t<div class=\"e-con-inner\">\n\t\t\t\t<div class=\"elementor-element elementor-element-c2fc337 elementor-widget elementor-widget-spacer\" data-id=\"c2fc337\" data-element_type=\"widget\" data-widget_type=\"spacer.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t<div class=\"elementor-spacer\">\n\t\t\t<div class=\"elementor-spacer-inner\"><\/div>\n\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-17499e21 the7-e-sticky-row-yes e-flex e-con-boxed e-con e-parent\" data-id=\"17499e21\" data-element_type=\"container\" id=\"sub-nav-bar\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;,&quot;the7_sticky_row&quot;:&quot;yes&quot;,&quot;the7_sticky_row_offset&quot;:116,&quot;the7_sticky_row_offset_tablet_extra&quot;:60,&quot;the7_sticky_row_offset_tablet&quot;:50,&quot;the7_sticky_row_offset_mobile&quot;:60,&quot;the7_sticky_row_devices&quot;:[&quot;desktop&quot;,&quot;laptop&quot;,&quot;tablet_extra&quot;,&quot;tablet&quot;,&quot;mobile&quot;]}\">\n\t\t\t\t\t<div class=\"e-con-inner\">\n\t\t<div class=\"elementor-element elementor-element-6a84235f e-con-full e-flex e-con e-child\" data-id=\"6a84235f\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-c9baf7e elementor-widget elementor-widget-text-editor\" data-id=\"c9baf7e\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<p><a href=\"#section-diseas-overview\">Vue d\u2019ensemble de la maladie<\/a><\/p>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-2f6d2303 e-con-full e-flex e-con e-child\" data-id=\"2f6d2303\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-2ad05b4d elementor-widget elementor-widget-text-editor\" data-id=\"2ad05b4d\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<p><a href=\"#aetiology-and-pathogenesis\">\u00c9tiologie et pathogen\u00e8se<\/a><\/p>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-f6d335f e-con-full e-flex e-con e-child\" data-id=\"f6d335f\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-1ccab512 elementor-widget elementor-widget-text-editor\" data-id=\"1ccab512\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<p><a href=\"#management-principles\">Principes de prise en charge<\/a><\/p>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-0b88976 e-con-full e-flex e-con e-parent\" data-id=\"0b88976\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t<div class=\"elementor-element elementor-element-211478f e-flex e-con-boxed e-con e-child\" data-id=\"211478f\" data-element_type=\"container\">\n\t\t\t\t\t<div class=\"e-con-inner\">\n\t\t\t\t<div class=\"elementor-element elementor-element-f04f473 elementor-widget elementor-widget-heading\" data-id=\"f04f473\" data-element_type=\"widget\" data-widget_type=\"heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h1 class=\"elementor-heading-title elementor-size-default\">\u00c0 propos des tumeurs desmo\u00efdes<\/h1>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-ca92b45 e-con-full section e-flex e-con e-parent\" data-id=\"ca92b45\" data-element_type=\"container\" id=\"section-diseas-overview\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t<div class=\"elementor-element elementor-element-811fdea e-flex e-con-boxed e-con e-child\" data-id=\"811fdea\" data-element_type=\"container\">\n\t\t\t\t\t<div class=\"e-con-inner\">\n\t\t<div class=\"elementor-element elementor-element-ff15afb e-con-full e-flex e-con e-child\" data-id=\"ff15afb\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-57d4d45 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"57d4d45\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h2 class=\"the7-heading-title elementor-size-default\">Vue d\u2019ensemble de la maladie<\/h2>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-ef6eb28 e-con-full e-flex e-con e-child\" data-id=\"ef6eb28\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-80d2ae5 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"80d2ae5\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h4 class=\"the7-heading-title elementor-size-default\">Une maladie infiltrante et agressive<sup>1<\/sup><\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-2cfcf7f elementor-widget-tablet__width-inherit [12:16 PM] Giovanni Ferraioli bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"2cfcf7f\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<ul><li>Les tumeurs desmo\u00efdes sont des tumeurs <strong>localement agressives<\/strong> des tissus mous potentiellement morbides, qui ont tendance \u00e0 infiltrer les structures environnantes et \u00e0 se propager le long des parois et des muscles<sup>1,2<\/sup>.<\/li><li>Parfois appel\u00e9es fibromatose agressive ou fibromatose desmo\u00efde<sup>3<\/sup>, ces tumeurs m\u00e9senchymateuses peuvent \u00eatre <strong>graves<\/strong>, <strong>invalidantes<\/strong> voire, dans de rares cas, entra\u00eener des <strong>l\u00e9sions potentiellement mortelles au niveau des organes<\/strong><sup>2<\/sup>.<\/li><li>Les tumeurs desmo\u00efdes peuvent <strong>infiltrer les tissus adjacents, comprimant ainsi les muscles, les nerfs et les vaisseaux<\/strong><sup>4,5<\/sup>.<\/li><\/ul>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-b951982 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"b951982\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h3 class=\"the7-heading-title elementor-size-default\">Symptomatologie et localisations tumorales<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-42360bd elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"42360bd\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h4 class=\"the7-heading-title elementor-size-default\">Les tumeurs desmo\u00efdes peuvent entra\u00eener des morbidit\u00e9s s\u00e9v\u00e8res et invalidantes<sup>2<\/sup>.<\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-e13da05 elementor-widget-tablet__width-inherit [12:16 PM] Giovanni Ferraioli bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"e13da05\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<ul><li>La symptomatologie associ\u00e9e aux tumeurs desmo\u00efdes varie en fonction de la localisation de la tumeur<sup>5,6<\/sup>.<\/li><li>Une \u00e9tude de cohorte prospective a montr\u00e9 que les tumeurs au niveau de la <strong>paroi thoracique<\/strong>, des <strong>membres sup\u00e9rieurs<\/strong>, et de la <strong>t\u00eate et du cou<\/strong> \u00e9taient associ\u00e9es \u00e0 des r\u00e9sultats d\u00e9favorables suite \u00e0 une intervention chirurgicale<sup>7<\/sup>.<\/li><\/ul>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-ddbc567 elementor-widget elementor-widget-the7_button_widget\" data-id=\"ddbc567\" data-element_type=\"widget\" data-widget_type=\"the7_button_widget.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<div class=\"elementor-button-wrapper\"><a href=\"https:\/\/www.desmoidtumours.eu\/fr\/experience-des-patients\/#disease-burden\" class=\"box-button elementor-button elementor-size-md\">En savoir plus sur la prise en charge des tumeurs desmo\u00efdes<\/a><\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-b7186b8 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"b7186b8\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<p>\n<table id=\"tablepress-5\" class=\"tablepress tablepress-id-5 first-tumors-table\">\n<thead>\n<tr class=\"row-1\">\n\t<th class=\"column-1\">LOCALISATION DE LA TUMEUR\u200b<\/th><th class=\"column-2\">FR\u00c9QUENCE ESTIM\u00c9E<sup>5<\/sup>\u200b<\/th><th class=\"column-3\">SYMPT\u00d4MES ET COMPLICATIONS FR\u00c9QUENTS<\/th>\n<\/tr>\n<\/thead>\n<tbody class=\"row-striping\">\n<tr class=\"row-2\">\n\t<td class=\"column-1\"><strong>Intra-abdominale<\/strong><br \/><small>(y compris le m\u00e9sent\u00e8re)<\/small><\/td><td class=\"column-2\"><strong>20%<\/strong><\/td><td class=\"column-3\">La compression peut entra\u00eener des douleurs, une cachexie, une sensation de malaise, une distension abdominale ou une occlusion des intestins ou de l\u2019uret\u00e8re<sup>5,8<\/sup><\/td>\n<\/tr>\n<tr class=\"row-3\">\n\t<td class=\"column-1\"><strong>Paroi abdominale<br \/>\n<\/strong><\/td><td class=\"column-2\"><strong>16%<\/strong><\/td><td class=\"column-3\">Les tumeurs de grande taille peuvent provoquer un \u00e9tirement des tissus, une compression des vaisseaux sanguins et un d\u00e9placement de l\u2019intestin ou de la vessie<sup>9<\/sup><br \/>\n<\/td>\n<\/tr>\n<tr class=\"row-4\">\n\t<td class=\"column-1\"><strong>Membres inf\u00e9rieurs<br \/>\n<\/strong><\/td><td class=\"column-2\"><strong>16%<\/strong><\/td><td class=\"column-3\">Mobilit\u00e9 r\u00e9duite, douleur, raideur musculaire ou d\u00e9formation<sup>5,10<\/sup><\/td>\n<\/tr>\n<tr class=\"row-5\">\n\t<td class=\"column-1\"><strong>Paroi thoracique<br \/>\n<\/strong><\/td><td class=\"column-2\"><strong>15%<\/strong><\/td><td class=\"column-3\">Dyspn\u00e9e<sup>5<\/sup>, dysphagie<sup>5<\/sup>, invasion pleurale<sup>8<\/sup>, atteinte de la colonne vert\u00e9brale<sup>11<\/sup> ou des c\u00f4tes<sup>12<\/sup>, \u00e9rosion osseuse<sup>12<\/sup> et douleur<sup>12<\/sup><\/td>\n<\/tr>\n<tr class=\"row-6\">\n\t<td class=\"column-1\"><strong>Membres sup\u00e9rieurs<\/strong><\/td><td class=\"column-2\"><strong>14%<\/strong><\/td><td class=\"column-3\">Mobilit\u00e9 restreinte, atteinte musculaire et ligamentaire, faiblesse des membres, d\u00e9formation ou douleur<sup>5,13<\/sup><\/td>\n<\/tr>\n<tr class=\"row-7\">\n\t<td class=\"column-1\"><strong>T\u00eate et cou<\/strong><\/td><td class=\"column-2\"><strong>8%<\/strong><\/td><td class=\"column-3\">Douleur, d\u00e9ficit neurologique, proximit\u00e9 des structures vitales, y compris risque de mortalit\u00e9 li\u00e9 au resserrement vasculaire ou des voies respiratoires<sup>14<\/sup><\/td>\n<\/tr>\n<tr class=\"row-8\">\n\t<td class=\"column-1\"><strong>Autre<\/strong><\/td><td class=\"column-2\"><strong>11%<\/strong><\/td><td class=\"column-3\">Sympt\u00f4mes et complications variables selon la localisation<sup>5,6<\/sup><\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<!-- #tablepress-5 from cache --><\/p>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-44ca57b elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"44ca57b\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<p><strong>La localisation des tumeurs desmo\u00efdes peut avoir un impact consid\u00e9rable sur la qualit\u00e9 de vie<\/strong><sup>15<\/sup>. Par exemple, une tumeur desmo\u00efde pr\u00e9sentant des excroissances en \u00ab\u00a0forme de vrille\u00a0\u00bb<sup>1<\/sup> enveloppant les <strong>nerfs<\/strong> peut \u00eatre associ\u00e9e \u00e0 des <strong>douleurs neuropathiques invalidantes<\/strong><sup>5,16<\/sup>.<\/p>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-b6179db elementor-widget elementor-widget-the7_button_widget\" data-id=\"b6179db\" data-element_type=\"widget\" data-widget_type=\"the7_button_widget.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<div class=\"elementor-button-wrapper\"><a href=\"https:\/\/www.desmoidtumours.eu\/fr\/experience-des-patients\/#disease-burden\" class=\"box-button elementor-button elementor-size-md\">En savoir plus sur le fardeau associ\u00e9 aux tumeurs desmo\u00efdes<\/a><\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-b75cde8 e-grid e-con-full e-con e-child\" data-id=\"b75cde8\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-12c3eb6 e-con-full e-flex e-con e-child\" data-id=\"12c3eb6\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-4e632bd elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"4e632bd\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h3 class=\"the7-heading-title elementor-size-default\">\u00c9volution de la maladie\u200b<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-be302d4 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"be302d4\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h4 class=\"the7-heading-title elementor-size-default\">Variable et impr\u00e9visible<sup>1<\/sup><\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-df99c5a elementor-widget-tablet__width-inherit [12:16 PM] Giovanni Ferraioli bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"df99c5a\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<ul><li>Les tumeurs desmo\u00efdes se caract\u00e9risent par une \u00e9volution variable et impr\u00e9visible en fonction de la localisation de la tumeur et de la morbidit\u00e9 associ\u00e9e<sup>1,5<\/sup>.<\/li><li>Environ 50&nbsp;% des tumeurs desmo\u00efdes pr\u00e9sentent une biologie agressive, et continuent de cro\u00eetre ou deviennent symptomatiques. La grande majorit\u00e9 des progressions (89&nbsp;%) sont survenues au cours des deux&nbsp;premi\u00e8res ann\u00e9es d\u2019observation<sup>4<\/sup>.<\/li><\/ul>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-2c74588 e-con-full e-flex e-con e-child\" data-id=\"2c74588\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-bcad1c9 content-align-center icon-vertical-align-center elementor-widget__width-inherit no-link icon-box-change-position-30 icon-box-vertical-align-top elementor-widget elementor-widget-the7_image_box_widget\" data-id=\"bcad1c9\" data-element_type=\"widget\" data-widget_type=\"the7_image_box_widget.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\n\t\t<div class=\"the7-box-wrapper the7-elementor-widget the7_image_box_widget-bcad1c9\">\t\t\t<div class=\"box-content-wrapper\">\n\t\t\t\t\t\t\t\t<div class=\"elementor-image-div \">\n\t\t\t\t\t<a class=\"post-thumbnail-rollover layzr-bg img-css-resize-wrapper\" ><img fetchpriority=\"high\" decoding=\"async\" width=\"1921\" height=\"1083\" src=\"data:image\/svg+xml,%3Csvg%20xmlns%3D&#039;http%3A%2F%2Fwww.w3.org%2F2000%2Fsvg&#039;%20viewBox%3D&#039;0%200%201921%201083&#039;%2F%3E\" class=\"attachment-full size-full preload-me aspect lazy lazy-load\" alt=\"\" sizes=\"(max-width: 1921px) 100vw, 1921px\" style=\"--ratio: 1921 \/ 1083;\" data-src=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2025\/04\/Springworks_charts_4FR.png\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2025\/04\/Springworks_charts_4FR.png 1921w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2025\/04\/Springworks_charts_4FR-300x169.png 300w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2025\/04\/Springworks_charts_4FR-1024x577.png 1024w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2025\/04\/Springworks_charts_4FR-768x433.png 768w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2025\/04\/Springworks_charts_4FR-1536x866.png 1536w\" \/><span class=\"the7-hover-icon\"><\/span><\/a>\t\t\t\t<\/div>\n\t\t\t\t\n\t\t\t\t<div class=\"box-content\">\n\t\t\t\t\t<h6 class=\"box-heading\"><a >\u00c9volution clinique des tumeurs desmo\u00efdes<\/a><\/h6><div class=\"box-description\">\u00c9labor\u00e9 \u00e0 partir du texte, r\u00e9f\u00e9rence&nbsp;17.<br>\n*&nbsp;10&nbsp;\u00e0&nbsp;28&nbsp;% des tumeurs desmo\u00efdes dispara\u00eetront\nspontan\u00e9ment sans traitement<sup>17<\/sup>.<\/div>\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-26d8756 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"26d8756\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h3 class=\"the7-heading-title elementor-size-default\">Incidence et facteurs de risque<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-eebac6b e-con-full e-flex e-con e-child\" data-id=\"eebac6b\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-275c1b7 no-link [12:16 PM] Giovanni Ferraioli bullet-custom   content-align-left icon-box-vertical-align-top icon-position-left icon-vertical-align-start elementor-widget elementor-widget-the7_icon_box_grid_widget\" data-id=\"275c1b7\" data-element_type=\"widget\" data-widget_type=\"the7_icon_box_grid_widget.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<div class=\"the7-box-grid-wrapper the7-elementor-widget loading-effect-none the7_icon_box_grid_widget-275c1b7\">\t\t\t<div class=\"dt-css-grid\">\n\t\t\t\t\t\t\t\t\t<div class=\"wf-cell shown\">\n\t\t\t\t\t\t<div class=\"the7-icon-box-grid\">\t\t\t\t\t\t\t<div class=\"box-content-wrapper\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<a class=\"elementor-icon-div\" aria-label=\"\u00c9pid\u00e9miologie\">\t\t\t\t\t\t\t\t\t\t<div class=\"elementor-icon\">\n\t\t\t\t\t\t\t\t\t\t\t<svg xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"186\" height=\"186\" viewBox=\"0 0 186 186\" fill=\"none\"><g clip-path=\"url(#clip0_316_1045)\"><g clip-path=\"url(#clip1_316_1045)\"><path opacity=\"0.3\" d=\"M173.66 12.22C157.36 -4.08 130.95 -4.08 114.65 12.22C108.36 18.51 104.56 26.3 103.13 34.43C103.09 34.62 103.03 34.75 102.99 34.93C98.6599 57.59 103.65 73.86 75.0599 102.46C46.4599 131.05 42.7899 123.43 19.2599 131.29L19.3399 131.38C15.2799 132.7 11.4299 134.86 8.19994 138.08C-2.73006 149.02 -2.73006 166.74 8.19994 177.67C19.1399 188.61 36.8599 188.61 47.7899 177.67C51.0199 174.44 53.1799 170.59 54.4999 166.53L54.5899 166.61C62.4499 143.07 54.8299 139.41 83.4199 110.81C112.01 82.21 128.29 87.2 150.95 82.88C151.14 82.85 151.26 82.78 151.44 82.74C159.58 81.31 167.38 77.5 173.66 71.21C189.96 54.92 189.96 28.5 173.66 12.2\" fill=\"#05C0DA\"><\/path><path d=\"M142.42 103.31V98.1601V121.49C142.42 123.04 143.68 124.3 145.23 124.3C146.78 124.3 148.04 123.04 148.04 121.49V97.7201C148.04 89.2301 144.71 82.3201 136.22 82.3201H126.66C121.66 82.3201 117.54 84.0501 115.62 86.9401C115.62 86.9401 113.96 89.6401 113.19 92.5201C117.59 96.2801 120.39 101.85 120.39 108.07V133.89C120.39 135.72 119.65 137.38 118.47 138.6V151.54H129.65H138.85L142.42 121.49V103.31Z\" fill=\"white\"><\/path><path d=\"M142.42 121.49V98.1601V103.31V121.49ZM142.42 121.49C142.42 123.04 143.68 124.3 145.23 124.3C146.78 124.3 148.04 123.04 148.04 121.49V97.7201C148.04 89.2301 144.71 82.3201 136.22 82.3201H126.66C121.66 82.3201 117.54 84.0501 115.62 86.9401C115.62 86.9401 113.96 89.6401 113.19 92.5201C117.59 96.2801 120.39 101.85 120.39 108.07V133.89C120.39 135.72 119.65 137.38 118.47 138.6V151.54H129.65M142.42 121.49L138.85 151.54H129.65M129.65 151.54V122.16\" stroke=\"#005EA7\" stroke-width=\"3.52\" stroke-miterlimit=\"10\"><\/path><path d=\"M65.44 133.89V108.07C65.44 101.85 68.24 96.28 72.64 92.52C71.83 90 70.29 87.63 70.27 87.61C68.36 84.72 64.23 82.99 59.24 82.99H49.68C41.19 82.99 37.86 89.9 37.86 98.39V122.16C37.86 123.71 39.12 124.97 40.67 124.97C42.22 124.97 43.48 123.71 43.48 122.16L47.05 151.6H56.25H69.12V139.87C66.95 138.74 65.45 136.49 65.45 133.88L65.44 133.89Z\" fill=\"white\"><\/path><path d=\"M43.48 122.16C43.48 123.71 42.22 124.97 40.67 124.97C39.12 124.97 37.86 123.71 37.86 122.16V98.39C37.86 89.9 41.19 82.99 49.68 82.99H59.24C64.23 82.99 68.36 84.72 70.27 87.61C70.29 87.63 71.83 90 72.64 92.52C68.24 96.28 65.44 101.85 65.44 108.07V133.89L65.45 133.88C65.45 136.49 66.95 138.74 69.12 139.87V151.6H56.25M43.48 122.16V98.16M43.48 122.16L47.05 151.6H56.25M56.25 151.6V122.16\" stroke=\"#005EA7\" stroke-width=\"3.52\" stroke-miterlimit=\"10\"><\/path><path d=\"M105 151.61V107.81V133.88C105 135.65 106.44 137.09 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stroke-width=\"3.52\" stroke-miterlimit=\"10\"><\/path><path d=\"M77.47 54.95C82.01 54.95 86.28 48.27 86.28 43.09C86.28 38.23 82.33 34.28 77.47 34.28C72.61 34.28 68.66 38.24 68.66 43.09C68.66 48.27 72.93 54.95 77.47 54.95Z\" fill=\"white\" stroke=\"#005EA7\" stroke-width=\"3.52\" stroke-miterlimit=\"10\"><\/path><path d=\"M107.55 54.95C112.09 54.95 116.36 48.27 116.36 43.09C116.36 38.23 112.41 34.28 107.55 34.28C102.69 34.28 98.74 38.24 98.74 43.09C98.74 48.27 103.01 54.95 107.55 54.95Z\" fill=\"white\" stroke=\"#005EA7\" stroke-width=\"3.52\" stroke-miterlimit=\"10\"><\/path><path d=\"M54.46 75.6201C59 75.6201 63.27 68.9401 63.27 63.7601C63.27 58.9001 59.32 54.9501 54.46 54.9501C49.6 54.9501 45.65 58.9101 45.65 63.7601C45.65 68.9401 49.92 75.6201 54.46 75.6201Z\" fill=\"white\" stroke=\"#005EA7\" stroke-width=\"3.52\" stroke-miterlimit=\"10\"><\/path><path d=\"M132.18 74.7C136.72 74.7 140.99 68.02 140.99 62.84C140.99 57.98 137.04 54.03 132.18 54.03C127.32 54.03 123.37 57.99 123.37 62.84C123.37 68.02 127.64 74.7 132.18 74.7Z\" fill=\"white\" stroke=\"#005EA7\" stroke-width=\"3.52\" stroke-miterlimit=\"10\"><\/path><\/g><\/g><defs><clipPath id=\"clip0_316_1045\"><rect width=\"186\" height=\"186\" fill=\"white\"><\/rect><\/clipPath><clipPath id=\"clip1_316_1045\"><rect width=\"185.89\" height=\"185.89\" fill=\"white\"><\/rect><\/clipPath><\/defs><\/svg>\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t<\/a>\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"box-content\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<h5 class=\"box-heading\">\n\t\t\t\t\t\t\t\t\t\t\t<a aria-label=\"\u00c9pid\u00e9miologie\">\t\t\t\t\t\t\t\t\t\t\t\t\u00c9pid\u00e9miologie\t\t\t\t\t\t\t\t\t\t\t<\/a>\t\t\t\t\t\t\t\t\t\t<\/h5>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"box-description\"><p>L\u2019incidence annuelle de la tumeur desmo\u00efde est d\u2019environ <strong>5&nbsp;cas pour 1&nbsp;million d\u2019habitants<\/strong><sup>18<\/sup>.<\/p>\n<p>L\u2019Orphanet Report Series indique une incidence annuelle moyenne de trois&nbsp;cas de tumeurs desmo\u00efdes par million de personnes-ann\u00e9es en Europe<sup>17<\/sup>.<\/p>\n<p>Les \u00e9tudes \u00e9pid\u00e9miologiques r\u00e9v\u00e8lent que la plupart des cas de tumeurs desmo\u00efdes surviennent <strong>chez les personnes \u00e2g\u00e9es de 20&nbsp;\u00e0 44&nbsp;ans<\/strong><sup>17<\/sup>.<\/p>\n<ul>\n \t<li>Les tumeurs desmo\u00efdes sont rares chez les jeunes et les personnes \u00e2g\u00e9es<sup>19<\/sup>.<\/li>\n<li>L\u2019estimation de l\u2019incidence a augment\u00e9 ces derni\u00e8res ann\u00e9es, ce qui peut \u00eatre li\u00e9 \u00e0 une am\u00e9lioration des techniques de diagnostic<sup>17<\/sup>.<\/li>\n<\/ul><\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t<div class=\"wf-cell shown\">\n\t\t\t\t\t\t<div class=\"the7-icon-box-grid\">\t\t\t\t\t\t\t<div class=\"box-content-wrapper\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<a class=\"elementor-icon-div\" aria-label=\"Patients de sexe f\u00e9minin\">\t\t\t\t\t\t\t\t\t\t<div class=\"elementor-icon\">\n\t\t\t\t\t\t\t\t\t\t\t<svg xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"186\" height=\"186\" viewBox=\"0 0 186 186\" fill=\"none\"><g clip-path=\"url(#clip0_313_829)\"><g clip-path=\"url(#clip1_313_829)\"><path opacity=\"0.3\" d=\"M173.66 12.22C157.36 -4.08 130.95 -4.08 114.65 12.22C108.36 18.51 104.56 26.3 103.13 34.43C103.09 34.62 103.03 34.75 102.99 34.93C98.6599 57.59 103.65 73.86 75.0599 102.46C46.4599 131.05 42.7899 123.43 19.2599 131.29L19.3399 131.38C15.2799 132.7 11.4299 134.86 8.19994 138.08C-2.73006 149.02 -2.73006 166.74 8.19994 177.67C19.1399 188.61 36.8599 188.61 47.7899 177.67C51.0199 174.44 53.1799 170.59 54.4999 166.53L54.5899 166.61C62.4499 143.07 54.8299 139.41 83.4199 110.81C112.01 82.21 128.29 87.2 150.95 82.88C151.14 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142.87V134.22C136.45 115.57 132.86 103.15 115.33 103.15H115.34Z\" stroke=\"#005EA7\" stroke-width=\"3.52\" stroke-miterlimit=\"10\"><\/path><path d=\"M68.97 94.04C68.97 95.31 70 96.35 71.28 96.35H115.3C116.57 96.35 117.61 95.32 117.61 94.04V64.88C117.62 64.46 117.93 54.55 111.14 47.48C106.83 43 100.72 40.72 92.99 40.72C85.26 40.72 79.2 43 75 47.49C68.37 54.58 68.96 64.52 68.98 64.78V94.03L68.97 94.04ZM92.98 92.6C84.11 92.6 78.46 81.92 77.44 70.32C77.25 68.12 77.14 65.94 77.15 63.84V57.59C80.76 59.88 91.22 65.41 108.75 66.08C108.71 67.48 108.64 68.89 108.51 70.32C107.5 81.91 101.85 92.6 92.97 92.6H92.98Z\" stroke=\"#005EA7\" stroke-width=\"3.52\" stroke-miterlimit=\"10\"><\/path><\/g><\/g><defs><clipPath id=\"clip0_313_829\"><rect width=\"186\" height=\"186\" fill=\"white\"><\/rect><\/clipPath><clipPath id=\"clip1_313_829\"><rect width=\"185.89\" height=\"185.89\" fill=\"white\"><\/rect><\/clipPath><\/defs><\/svg>\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t<\/a>\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"box-content\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<h5 class=\"box-heading\">\n\t\t\t\t\t\t\t\t\t\t\t<a aria-label=\"Patients de sexe f\u00e9minin\">\t\t\t\t\t\t\t\t\t\t\t\tPatients de sexe f\u00e9minin\t\t\t\t\t\t\t\t\t\t\t<\/a>\t\t\t\t\t\t\t\t\t\t<\/h5>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"box-description\">\n<p>Le ratio femmes\/hommes est d\u2019environ 2&nbsp;\u00e0&nbsp;3&nbsp;pour&nbsp;1<sup>20<\/sup>.<\/p>\n<ul>\n<li>La grossesse peut favoriser le d\u00e9veloppement des tumeurs desmo\u00efdes<sup>4<\/sup>.<\/li>\n<\/ul><\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t<div class=\"wf-cell shown\">\n\t\t\t\t\t\t<div class=\"the7-icon-box-grid\">\t\t\t\t\t\t\t<div class=\"box-content-wrapper\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<a class=\"elementor-icon-div\" aria-label=\"Ant\u00e9c\u00e9dents de traumatisme\">\t\t\t\t\t\t\t\t\t\t<div class=\"elementor-icon\">\n\t\t\t\t\t\t\t\t\t\t\t<svg xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"186\" height=\"186\" viewBox=\"0 0 186 186\" fill=\"none\"><g clip-path=\"url(#clip0_316_1153)\"><g clip-path=\"url(#clip1_316_1153)\"><path opacity=\"0.3\" d=\"M173.66 12.22C157.36 -4.08 130.95 -4.08 114.65 12.22C108.36 18.51 104.56 26.3 103.13 34.43C103.09 34.62 103.03 34.75 102.99 34.93C98.6599 57.59 103.65 73.86 75.0599 102.46C46.4599 131.05 42.7899 123.43 19.2599 131.29L19.3399 131.38C15.2799 132.7 11.4299 134.86 8.19994 138.08C-2.73006 149.02 -2.73006 166.74 8.19994 177.67C19.1399 188.61 36.8599 188.61 47.7899 177.67C51.0199 174.44 53.1799 170.59 54.4999 166.53L54.5899 166.61C62.4499 143.07 54.8299 139.41 83.4199 110.81C112.01 82.21 128.29 87.2 150.95 82.88C151.14 82.85 151.26 82.78 151.44 82.74C159.58 81.31 167.38 77.5 173.66 71.21C189.96 54.92 189.96 28.5 173.66 12.2\" fill=\"#05C0DA\"><\/path><path d=\"M85.5701 50.17L36.1801 135.71C32.9001 141.38 37.0001 148.48 43.5501 148.48H142.33C148.88 148.48 152.98 141.39 149.7 135.71L100.31 50.17C97.0301 44.5 88.8401 44.5 85.5701 50.17Z\" fill=\"white\" stroke=\"#005EA7\" stroke-width=\"3.52\" stroke-miterlimit=\"10\"><\/path><path d=\"M92.94 70.02V118.18\" stroke=\"#005EA7\" stroke-width=\"6\" stroke-miterlimit=\"10\"><\/path><path d=\"M92.94 128.29V135.67\" stroke=\"#005EA7\" stroke-width=\"5.02\" stroke-miterlimit=\"10\"><\/path><\/g><\/g><defs><clipPath id=\"clip0_316_1153\"><rect width=\"186\" height=\"186\" fill=\"white\"><\/rect><\/clipPath><clipPath id=\"clip1_316_1153\"><rect width=\"185.89\" height=\"185.89\" fill=\"white\"><\/rect><\/clipPath><\/defs><\/svg>\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t<\/a>\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"box-content\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<h5 class=\"box-heading\">\n\t\t\t\t\t\t\t\t\t\t\t<a aria-label=\"Ant\u00e9c\u00e9dents de traumatisme\">\t\t\t\t\t\t\t\t\t\t\t\tAnt\u00e9c\u00e9dents de traumatisme\t\t\t\t\t\t\t\t\t\t\t<\/a>\t\t\t\t\t\t\t\t\t\t<\/h5>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"box-description\"><p><strong>Environ 25&nbsp;%<\/strong> des patients atteints de tumeurs desmo\u00efdes pr\u00e9sentent des ant\u00e9c\u00e9dents de traumatisme<sup>5<\/sup>.\n<\/p>\n<ul>\n<li>Une blessure ou une intervention chirurgicale peut augmenter le risque<sup>21<\/sup>.<\/li>\n<\/ul><\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t<div class=\"wf-cell shown\">\n\t\t\t\t\t\t<div class=\"the7-icon-box-grid\">\t\t\t\t\t\t\t<div class=\"box-content-wrapper\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<a class=\"elementor-icon-div\" aria-label=\"Mutations APC (g\u00e8ne de la polypose ad\u00e9nomateuse coli)\">\t\t\t\t\t\t\t\t\t\t<div class=\"elementor-icon\">\n\t\t\t\t\t\t\t\t\t\t\t<svg xmlns=\"http:\/\/www.w3.org\/2000\/svg\" id=\"Layer_2\" viewBox=\"0 0 185.89 185.89\"><defs><style>      .cls-1 {        fill: #05c0da;        opacity: .3;      }      .cls-2 {        fill: none;      }      .cls-2, .cls-3 {        stroke: #005ea7;        stroke-miterlimit: 10;        stroke-width: 3.52px;      }      .cls-4, .cls-3 {        fill: #fff;      }    <\/style><\/defs><g id=\"Layer_1-2\" data-name=\"Layer_1\"><g><path class=\"cls-1\" d=\"M173.66,12.22c-16.3-16.3-42.71-16.3-59.01,0-6.29,6.29-10.09,14.08-11.52,22.21-.04.19-.1.32-.14.5-4.33,22.66.66,38.93-27.93,67.53-28.6,28.59-32.27,20.97-55.8,28.83l.08.09c-4.06,1.32-7.91,3.48-11.14,6.7-10.93,10.94-10.93,28.66,0,39.59,10.94,10.94,28.66,10.94,39.59,0,3.23-3.23,5.39-7.08,6.71-11.14l.09.08c7.86-23.54.24-27.2,28.83-55.8,28.59-28.6,44.87-23.61,67.53-27.93.19-.03.31-.1.49-.14,8.14-1.43,15.94-5.24,22.22-11.53,16.3-16.29,16.3-42.71,0-59.01\"><\/path><g><polygon class=\"cls-4\" points=\"85.2 131.47 66.82 133.65 93.64 59.95 103.59 61.85 85.2 131.47\"><\/polygon><g><path class=\"cls-2\" d=\"M43.79,115.19c-10.18-4.36-22.07,0-29.87,7.81\"><\/path><path class=\"cls-2\" d=\"M131.72,77.5c24,9.37,35.59-2.09,35.59-2.09\"><\/path><path class=\"cls-3\" d=\"M69.59,139.56c-1.05-4.59-3.22-9.43-6.71-14.52-25.41-36.92-10.46-51.92-3.66-58.78h0c8.19-8.18,22.16-9.1,35.98-4.34\"><\/path><path class=\"cls-2\" d=\"M59.8,168.88c8.06-8.07,12.39-17.91,9.79-29.32\"><\/path><\/g><path class=\"cls-3\" d=\"M101.82,39.51c.82,9.17,4.64,19.17,10.81,28.13,25.41,36.92,10.46,51.92,3.66,58.78h0c-8.19,8.18-22.16,9.1-35.98,4.34\"><\/path><path class=\"cls-2\" d=\"M108.69,16.79c-5.59,5.59-7.67,13.76-6.87,22.73\"><\/path><g><line class=\"cls-2\" x1=\"71\" y1=\"79.6\" x2=\"104.5\" y2=\"113.09\"><\/line><line class=\"cls-2\" x1=\"121.25\" y1=\"29.35\" x2=\"154.75\" y2=\"62.85\"><\/line><line class=\"cls-2\" x1=\"121.25\" y1=\"50.29\" x2=\"133.81\" y2=\"62.85\"><\/line><line class=\"cls-2\" x1=\"23.76\" y1=\"132.84\" x2=\"52.26\" y2=\"161.34\"><\/line><line class=\"cls-2\" x1=\"41.69\" y1=\"129.84\" x2=\"54.26\" y2=\"142.4\"><\/line><line class=\"cls-2\" x1=\"87.75\" y1=\"75.41\" x2=\"108.69\" y2=\"96.34\"><\/line><line class=\"cls-2\" x1=\"66.82\" y1=\"96.34\" x2=\"87.75\" y2=\"117.28\"><\/line><\/g><\/g><\/g><\/g><\/svg>\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t<\/a>\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"box-content\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<h5 class=\"box-heading\">\n\t\t\t\t\t\t\t\t\t\t\t<a aria-label=\"Mutations APC (g\u00e8ne de la polypose ad\u00e9nomateuse coli)\">\t\t\t\t\t\t\t\t\t\t\t\tMutations APC (g\u00e8ne de la polypose ad\u00e9nomateuse coli)\t\t\t\t\t\t\t\t\t\t\t<\/a>\t\t\t\t\t\t\t\t\t\t<\/h5>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"box-description\"><p>Les <strong>caract\u00e9ristiques ph\u00e9notypiques<\/strong> sont corr\u00e9l\u00e9es \u00e0 la position de la mutation APC par rapport \u00e0 certains codons<sup>22<\/sup>.\n<\/p>\n<ul>\n \t<li>Les patients atteints de polypose ad\u00e9nomateuse familiale (PAF) pr\u00e9sentent un risque <strong>1&nbsp;000&nbsp;fois plus \u00e9lev\u00e9<\/strong> de d\u00e9velopper des tumeurs desmo\u00efdes par rapport \u00e0 la population g\u00e9n\u00e9rale<sup>20<\/sup>.<\/li>\n<\/ul><\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t<\/div>\n\t\t<\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-28f2403 e-con-full section e-flex e-con e-parent\" data-id=\"28f2403\" data-element_type=\"container\" id=\"aetiology-and-pathogenesis\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t<div class=\"elementor-element elementor-element-b7c7805 e-flex e-con-boxed e-con e-child\" data-id=\"b7c7805\" data-element_type=\"container\">\n\t\t\t\t\t<div class=\"e-con-inner\">\n\t\t<div class=\"elementor-element elementor-element-e7ef271 e-con-full e-flex e-con e-child\" data-id=\"e7ef271\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-f727336 e-con-full e-flex e-con e-child\" data-id=\"f727336\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-5f1a32b elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"5f1a32b\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h2 class=\"the7-heading-title elementor-size-default\">\u00c9tiologie et pathogen\u00e8se des tumeurs desmo\u00efdes <\/h2>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-1e29fb4 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"1e29fb4\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h4 class=\"the7-heading-title elementor-size-default\">La compr\u00e9hension de la pathogen\u00e8se mol\u00e9culaire pr\u00e9sente un int\u00e9r\u00eat consid\u00e9rable dans cette maladie en raison de son \u00e9volution clinique tr\u00e8s variable<sup>19<\/sup>.<\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-40f7dc0 elementor-widget-tablet__width-inherit [12:16 PM] Giovanni Ferraioli bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"40f7dc0\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<p>L\u2019\u00e9tiologie des tumeurs desmo\u00efdes est inconnue. Cependant, l\u2019identification d\u2019alt\u00e9rations chromosomiques clonales dans une proportion significative des cas confirme la nature n\u00e9oplasique de ces tumeurs<sup>19<\/sup>.<\/p><ul><li>La pathogen\u00e8se des tumeurs desmo\u00efdes serait li\u00e9e \u00e0 un traumatisme<sup>4<\/sup>.<ul><li>L\u2019intervention chirurgicale peut parfois favoriser la croissance de la tumeur<sup>4<\/sup>.<\/li><li>Les facteurs de croissance lib\u00e9r\u00e9s apr\u00e8s une intervention chirurgicale au cours de la phase initiale de cicatrisation pourraient transmettre des signaux qui favorisent l\u2019activation de la \u03b2-cat\u00e9nine<sup>4<\/sup>.<\/li><\/ul><\/li><li>La grossesse peut favoriser l\u2019apparition de tumeurs desmo\u00efdes, non seulement en raison d\u2019influences hormonales, mais aussi en raison de la lib\u00e9ration syst\u00e9mique de facteurs de croissance<sup>4<\/sup>.<\/li><li>Deux voies ont \u00e9t\u00e9 impliqu\u00e9es dans la voie canonique <strong>Wnt\/<b>\u03b2<\/b>-cat\u00e9nine\/APC<\/strong>, dans laquelle les mutations CTNNB1 et APC entra\u00eenent une accumulation de \u03b2-cat\u00e9nine, et la <strong>voie Notch<\/strong>, dans laquelle deux clivages surviennent au niveau des r\u00e9cepteurs Notch <sup>17<\/sup>.<ul><li>On pense que l\u2019intercommunication potentielle entre les voies Notch et Wnt, ainsi que l\u2019activation de la voie Notch r\u00e9sultant d\u2019une d\u00e9r\u00e9gulation de la voie Wnt, sont impliqu\u00e9es dans la pathogen\u00e8se des tumeurs desmo\u00efdes<sup>17<\/sup>.<\/li><\/ul><\/li><\/ul>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-2d5792b e-grid e-con-full e-con e-child\" data-id=\"2d5792b\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-3a76b96 no-link icon-box-change-position-30 bord-img-8 content-align-left icon-vertical-align-start elementor-widget elementor-widget-the7_image_box_widget\" data-id=\"3a76b96\" data-element_type=\"widget\" data-widget_type=\"the7_image_box_widget.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\n\t\t<div class=\"the7-box-wrapper the7-elementor-widget the7_image_box_widget-3a76b96\">\t\t\t<div class=\"box-content-wrapper\">\n\t\t\t\t\t\t\t\t<div class=\"elementor-image-div \">\n\t\t\t\t\t<a class=\"post-thumbnail-rollover layzr-bg img-css-resize-wrapper\" ><img decoding=\"async\" width=\"508\" height=\"696\" src=\"data:image\/svg+xml,%3Csvg%20xmlns%3D&#039;http%3A%2F%2Fwww.w3.org%2F2000%2Fsvg&#039;%20viewBox%3D&#039;0%200%20508%20696&#039;%2F%3E\" class=\"attachment-full size-full preload-me aspect lazy lazy-load\" alt=\"\" sizes=\"(max-width: 508px) 100vw, 508px\" style=\"--ratio: 508 \/ 696;\" data-src=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Nuclear-accumulation-of-beta-catenin.png\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Nuclear-accumulation-of-beta-catenin.png 508w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Nuclear-accumulation-of-beta-catenin-219x300.png 219w\" \/><span class=\"the7-hover-icon\"><\/span><\/a>\t\t\t\t<\/div>\n\t\t\t\t\n\t\t\t\t<div class=\"box-content\">\n\t\t\t\t\t<h6 class=\"box-heading\"><a >Accumulation nucl\u00e9aire de \u03b2-cat\u00e9nine<\/a><\/h6><div class=\"box-description\"><ul>\n \t<li>On a pu observer que la d\u00e9r\u00e9gulation de la voie Wnt\/\u03b2-cat\u00e9nine \u00e9tait impliqu\u00e9e dans la pathogen\u00e8se des tumeurs desmo\u00efdes<sup>2,23<\/sup>.<\/li>\n \t<li>L\u2019accumulation nucl\u00e9aire de \u03b2-cat\u00e9nine et la transcription\ndu g\u00e8ne en aval constitue un facteur cl\u00e9 de la prolif\u00e9ration des cellules de tumeurs desmo\u00efdes<sup>2,24<\/sup>.<\/li>\n \t<li>L\u2019accumulation nucl\u00e9aire de \u03b2-cat\u00e9nine peut \u00eatre provoqu\u00e9e par<sup>2,24<\/sup>&nbsp;:\n<ul>\n \t<li>des mutations activatrices du g\u00e8ne de la \u03b2-cat\u00e9nine&nbsp;<em>CTNNB1<\/em><\/li>\n \t<li>des mutations inactivantes du r\u00e9gulateur n\u00e9gatif <em>APC<\/em>, souvent li\u00e9es aux cas de polypose ad\u00e9nomateuse familiale (PAF)<\/li>\n<\/ul>\n<\/li>\n<\/ul><\/div>\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-d935d19 no-link icon-box-change-position-30 bord-img-8 content-align-left icon-vertical-align-start elementor-widget elementor-widget-the7_image_box_widget\" data-id=\"d935d19\" data-element_type=\"widget\" data-widget_type=\"the7_image_box_widget.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\n\t\t<div class=\"the7-box-wrapper the7-elementor-widget the7_image_box_widget-d935d19\">\t\t\t<div class=\"box-content-wrapper\">\n\t\t\t\t\t\t\t\t<div class=\"elementor-image-div \">\n\t\t\t\t\t<a class=\"post-thumbnail-rollover layzr-bg img-css-resize-wrapper\" ><img decoding=\"async\" width=\"512\" height=\"696\" src=\"data:image\/svg+xml,%3Csvg%20xmlns%3D&#039;http%3A%2F%2Fwww.w3.org%2F2000%2Fsvg&#039;%20viewBox%3D&#039;0%200%20512%20696&#039;%2F%3E\" class=\"attachment-full size-full preload-me aspect lazy lazy-load\" alt=\"\" sizes=\"(max-width: 512px) 100vw, 512px\" style=\"--ratio: 512 \/ 696;\" data-src=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Notch-activation-by-gamma-secretase-copy-1.jpg\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Notch-activation-by-gamma-secretase-copy-1.jpg 512w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Notch-activation-by-gamma-secretase-copy-1-221x300.jpg 221w\" \/><span class=\"the7-hover-icon\"><\/span><\/a>\t\t\t\t<\/div>\n\t\t\t\t\n\t\t\t\t<div class=\"box-content\">\n\t\t\t\t\t<h6 class=\"box-heading\"><a >L\u2019activation de Notch par la \u03b3-s\u00e9cr\u00e9tase<\/a><\/h6><div class=\"box-description\"><ul>\n \t<li>La voie Notch peut \u00eatre active dans les tumeurs desmo\u00efdes<sup>25<\/sup>.<\/li>\n \t<li>Lorsqu\u2019elle est d\u00e9r\u00e9gul\u00e9e, la voie Notch peut activer d\u2019autres voies qui contribuent \u00e0 la croissance tumorale<sup>25<\/sup>.<\/li>\n \t<li>La signalisation du r\u00e9cepteur Notch n\u00e9cessite une activation prot\u00e9olytique par l\u2019enzyme \u03b3-s\u00e9cr\u00e9tase (gamma s\u00e9cr\u00e9tase)<sup>25<\/sup>.<\/li>\n \t<li>Le clivage de la \u03b3-s\u00e9cr\u00e9tase lib\u00e8re le domaine intracellulaire Notch (Notch intracellular domain, NICD), qui se d\u00e9place vers le noyau et active la transcription g\u00e9nique<sup>25<\/sup>.<\/li>\n<\/ul><\/div>\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-61bd4d0 no-link icon-box-change-position-30 bord-img-8 content-align-left icon-vertical-align-start elementor-widget elementor-widget-the7_image_box_widget\" data-id=\"61bd4d0\" data-element_type=\"widget\" data-widget_type=\"the7_image_box_widget.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\n\t\t<div class=\"the7-box-wrapper the7-elementor-widget the7_image_box_widget-61bd4d0\">\t\t\t<div class=\"box-content-wrapper\">\n\t\t\t\t\t\t\t\t<div class=\"elementor-image-div \">\n\t\t\t\t\t<a class=\"post-thumbnail-rollover layzr-bg img-css-resize-wrapper\" ><img decoding=\"async\" width=\"508\" height=\"696\" src=\"data:image\/svg+xml,%3Csvg%20xmlns%3D&#039;http%3A%2F%2Fwww.w3.org%2F2000%2Fsvg&#039;%20viewBox%3D&#039;0%200%20508%20696&#039;%2F%3E\" class=\"attachment-full size-full preload-me aspect lazy lazy-load\" alt=\"\" sizes=\"(max-width: 508px) 100vw, 508px\" style=\"--ratio: 508 \/ 696;\" data-src=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Patchway-crosstalk.jpg\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Patchway-crosstalk.jpg 508w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Patchway-crosstalk-219x300.jpg 219w\" \/><span class=\"the7-hover-icon\"><\/span><\/a>\t\t\t\t<\/div>\n\t\t\t\t\n\t\t\t\t<div class=\"box-content\">\n\t\t\t\t\t<h6 class=\"box-heading\"><a >Intercommunications des voies<\/a><\/h6><div class=\"box-description\"><ul>\n \t<li>Il est possible que les intercommunications entre les voies Wnt\/\u03b2-cat\u00e9nine et Notch contribuent \u00e0 la pathogen\u00e8se des tumeurs desmo\u00efdes<sup>17<\/sup>.<\/li>\n<\/ul><\/div>\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-fed41d1 elementor-widget elementor-widget-menu-anchor\" data-id=\"fed41d1\" data-element_type=\"widget\" data-widget_type=\"menu-anchor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t<div class=\"elementor-menu-anchor\" id=\"rcidive\"><\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-b80d6e9 e-con-full e-flex e-con e-child\" data-id=\"b80d6e9\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-2c29233 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"2c29233\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h3 class=\"the7-heading-title elementor-size-default\">Risque de r\u00e9cidive<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-57f5ab6 elementor-widget-tablet__width-inherit bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"57f5ab6\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<ul><li>Les taux de r\u00e9cidive peuvent \u00eatre influenc\u00e9s par <strong>la localisation de la tumeur<\/strong><sup>17<\/sup>&nbsp;:<ul><li>Les tumeurs au niveau des extr\u00e9mit\u00e9s sont localement agressives et pr\u00e9sentent des taux de r\u00e9cidive allant de 24&nbsp;% \u00e0 77&nbsp;%.<\/li><li>Les taux de r\u00e9cidive locale des tumeurs intra-abdominales chez les patients atteints de PAF sont plus \u00e9lev\u00e9s que ceux des tumeurs extra-abdominales, et seraient compris entre 57&nbsp;% et 86&nbsp;%.<\/li><\/ul><\/li><\/ul>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-1110ecd elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"1110ecd\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h4 class=\"the7-heading-title elementor-size-default\">Il existe un risque \u00e9lev\u00e9 de r\u00e9cidive locale suite \u00e0 une intervention chirurgicale<sup>17<\/sup><\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-10bb62a elementor-widget-tablet__width-inherit bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"10bb62a\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<ul><li>Les taux de r\u00e9cidive peuvent \u00eatre exacerb\u00e9s par des <strong>traumatismes<\/strong>, comme les traumatismes li\u00e9s \u00e0 une <strong>intervention chirurgicale<\/strong>, et varient d\u2019environ 25% a 60% en 5 ans<sup>17<\/sup>.<ul><li>L\u2019invasion des vaisseaux et des nerfs principaux, ainsi que la qualit\u00e9 des marges chirurgicales sont des facteurs cl\u00e9s du taux \u00e9lev\u00e9 de r\u00e9cidive postop\u00e9ratoire<sup>26<\/sup>.<\/li><li>Malheureusement, m\u00eame lorsque les marges chirurgicales sont exemptes de tumeur, les <strong>taux de r\u00e9cidive sont \u00e9lev\u00e9s<\/strong><sup>27<\/sup>.<\/li><\/ul><\/li><\/ul><p>Les facteurs de croissance lib\u00e9r\u00e9s au cours de la phase initiale de cicatrisation postop\u00e9ratoire pourraient favoriser l\u2019activation de la \u03b2-cat\u00e9nine<sup>4<\/sup>.<\/p><ul><li>Le fait que certaines tumeurs aient r\u00e9cidiv\u00e9 apr\u00e8s une intervention chirurgicale, mais se soient ensuite stabilis\u00e9es sans traitement, sugg\u00e8re que les facteurs de croissance lib\u00e9r\u00e9s apr\u00e8s l\u2019intervention chirurgicale pourraient avoir favoris\u00e9 la r\u00e9cidive de tumeurs qui seraient par ailleurs rest\u00e9es indolentes<sup>4<\/sup>.<\/li><li>La mutation sp\u00e9cifique S45F du g\u00e8ne CTNNB1 semble \u00eatre associ\u00e9e \u00e0 une survie sans r\u00e9cidive moins bonne apr\u00e8s une intervention chirurgicale<sup>28<\/sup>.<ul><li>La survie sans r\u00e9cidive \u00e0 5\u00a0ans \u00e9tait significativement plus faible chez les patients atteints de tumeurs desmo\u00efdes avec mutation S45F (P&lt; 0,0001) par rapport aux patients atteints de tumeurs avec mutation T41A ou ne pr\u00e9sentant pas de mutation<sup>29<\/sup>.<\/li><\/ul><\/li><\/ul>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-577adfa e-con-full e-flex e-con e-child\" data-id=\"577adfa\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-1a2357c elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"1a2357c\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<p>\n<table id=\"tablepress-6\" class=\"tablepress tablepress-id-6 tumors-table\">\n<thead>\n<tr class=\"row-1\">\n\t<th class=\"column-1\">MUTATION<\/th><th class=\"column-2\">SURVIE SANS R\u00c9CIDIVE ESTIM\u00c9E (IC \u00c0 95\u00a0%)<\/th>\n<\/tr>\n<\/thead>\n<tbody class=\"row-striping\">\n<tr class=\"row-2\">\n\t<td class=\"column-1\">45F<\/td><td class=\"column-2\">23% (10%-40%)<\/td>\n<\/tr>\n<tr class=\"row-3\">\n\t<td class=\"column-1\">41A<\/td><td class=\"column-2\">57% (43%-69%)<\/td>\n<\/tr>\n<tr class=\"row-4\">\n\t<td class=\"column-1\"><i>CTNNB1<\/i> non mut\u00e9<\/td><td class=\"column-2\">65% (38%-83%)<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<!-- #tablepress-6 from cache --><\/p>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-b740583 e-con-full e-flex e-con e-child\" data-id=\"b740583\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t<div class=\"elementor-element elementor-element-62e00f8 e-con-full e-flex e-con e-child\" data-id=\"62e00f8\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-991e6fd e-con-full e-flex e-con e-child\" data-id=\"991e6fd\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-a7fc8b0 no-link icon-box-change-position-50 content-align-left icon-box-vertical-align-top icon-vertical-align-start elementor-widget elementor-widget-the7_image_box_widget\" data-id=\"a7fc8b0\" data-element_type=\"widget\" data-widget_type=\"the7_image_box_widget.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\n\t\t<div class=\"the7-box-wrapper the7-elementor-widget the7_image_box_widget-a7fc8b0\">\t\t\t<div class=\"box-content-wrapper\">\n\t\t\t\t\t\t\t\t<div class=\"elementor-image-div \">\n\t\t\t\t\t<a class=\"post-thumbnail-rollover layzr-bg img-css-resize-wrapper\" ><img decoding=\"async\" width=\"1024\" height=\"577\" src=\"data:image\/svg+xml,%3Csvg%20xmlns%3D&#039;http%3A%2F%2Fwww.w3.org%2F2000%2Fsvg&#039;%20viewBox%3D&#039;0%200%201024%20577&#039;%2F%3E\" class=\"attachment-large size-large preload-me aspect lazy lazy-load\" alt=\"\" sizes=\"(max-width: 1024px) 100vw, 1024px\" style=\"--ratio: 1024 \/ 577;\" data-src=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2025\/04\/Springworks_charts_1FR-02-1-1024x577.png\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2025\/04\/Springworks_charts_1FR-02-1-1024x577.png 1024w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2025\/04\/Springworks_charts_1FR-02-1-300x169.png 300w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2025\/04\/Springworks_charts_1FR-02-1-768x433.png 768w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2025\/04\/Springworks_charts_1FR-02-1-1536x866.png 1536w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2025\/04\/Springworks_charts_1FR-02-1.png 1921w\" \/><span class=\"the7-hover-icon\"><\/span><\/a>\t\t\t\t<\/div>\n\t\t\t\t\n\t\t\t\t<div class=\"box-content\">\n\t\t\t\t\t<h6 class=\"box-heading\"><a >Les patients pr\u00e9sentant des mutations CTNNB1 au niveau du codon&nbsp;45 (45F) ont un risque de r\u00e9cidive postop\u00e9ratoire 3&nbsp;fois plus \u00e9lev\u00e9<sup>29<\/sup><\/a><\/h6><div class=\"box-description\">\u00c9labor\u00e9 \u00e0 partir de la figure&nbsp;2b, r\u00e9f\u00e9rence&nbsp;29.<\/div>\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-f072648 elementor-widget elementor-widget-text-editor\" data-id=\"f072648\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<p>Dans le cadre d\u2019une m\u00e9ta-analyse, la taille de la tumeur a \u00e9t\u00e9 identifi\u00e9e comme un m\u00e9diateur important dans l\u2019augmentation du risque de r\u00e9cidive chez les patients porteurs de la mutation <em>CTNNB1<\/em>\u00a0S45F<sup>30<\/sup>.<\/p>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-ac80b71 elementor-widget elementor-widget-the7_button_widget\" data-id=\"ac80b71\" data-element_type=\"widget\" data-widget_type=\"the7_button_widget.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<div class=\"elementor-button-wrapper\"><a href=\"https:\/\/www.desmoidtumours.eu\/fr\/prise-en-charge\/#surgery\" class=\"box-button elementor-button elementor-size-md\">Plus d\u2019informations sur les consid\u00e9rations chirurgicales<\/a><\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-8a390a1 e-con-full section e-flex e-con e-parent\" data-id=\"8a390a1\" data-element_type=\"container\" id=\"management-principles\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t<div class=\"elementor-element elementor-element-6ef8a6e e-flex e-con-boxed e-con e-child\" data-id=\"6ef8a6e\" data-element_type=\"container\">\n\t\t\t\t\t<div class=\"e-con-inner\">\n\t\t<div class=\"elementor-element elementor-element-d763c31 e-con-full e-flex e-con e-child\" data-id=\"d763c31\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-9b72f44 e-con-full e-flex e-con e-child\" data-id=\"9b72f44\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-fc8f101 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"fc8f101\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h2 class=\"the7-heading-title elementor-size-default\">Principes de prise en charge<\/h2>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-6a15008 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"6a15008\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h4 class=\"the7-heading-title elementor-size-default\">Il est imp\u00e9ratif de choisir avec soin la strat\u00e9gie de prise en charge de chaque patient atteint de tumeurs desmo\u00efdes afin d'optimiser le contr\u00f4le des tumeurs et d'am\u00e9liorer la qualit\u00e9 de vie<sup>18<\/sup>.<\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-99a3f81 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"99a3f81\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<p>Des efforts importants visant \u00e0 rationaliser la prise en charge de cette maladie ont \u00e9t\u00e9 entrepris au cours des dix derni\u00e8res ann\u00e9es<sup>18<\/sup>.<\/p><p>Actuellement, les besoins non satisfaits dans le cadre des tumeurs desmo\u00efdes incluent un diagnostic pr\u00e9coce et pr\u00e9cis et des traitements approuv\u00e9s indiqu\u00e9s pour les patients atteints de tumeurs desmo\u00efdes<sup>17<\/sup>.<\/p><p>Dans une \u00e9tude men\u00e9e au Memorial Sloan Kettering Cancer Center, 58&nbsp;% des patients atteints de tumeurs desmo\u00efdes sont pass\u00e9s d\u2019une surveillance active au traitement de premi\u00e8re intention sans progression de la tumeur sur le plan radiographique<sup>31\u2020<\/sup>.<\/p><p>Les objectifs du traitement ne doivent pas se concentrer uniquement sur des marqueurs cliniques, tels que la survie sans progression, mais \u00e9galement prendre en compte des crit\u00e8res d\u2019\u00e9valuation pertinents pour le patient, comme la r\u00e9duction du fardeau symptomatique sp\u00e9cifique des tumeurs desmo\u00efdes (par&nbsp;ex., la douleur) et leur impact sur la vie des patients, l\u2019am\u00e9lioration de leurs capacit\u00e9s pour effectuer les activit\u00e9s quotidiennes et leur qualit\u00e9 de vie globale<sup>17<\/sup>.<\/p>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-18e5c3d elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"18e5c3d\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h3 class=\"the7-heading-title elementor-size-default\">Identification pr\u00e9coce de la progression<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-057d790 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"057d790\" data-element_type=\"widget\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h4 class=\"the7-heading-title elementor-size-default\">L\u2019identification de la progression d\u2019une tumeur desmo\u00efde peut permettre l\u2019instauration plus pr\u00e9coce d\u2019un traitement appropri\u00e9. <\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-979b5f1 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"979b5f1\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<ul><li>Pour am\u00e9liorer les r\u00e9sultats des patients, les professionnels de sant\u00e9 devraient <strong>\u00e9valuer la progression le plus t\u00f4t possible<\/strong> avec au moins l\u2019un des \u00e9l\u00e9ments suivants&nbsp;:<\/li><\/ul>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-23d8ff6 e-con-fit e-flex e-con e-child\" data-id=\"23d8ff6\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-1467c94 no-link content-align-left icon-box-vertical-align-top icon-position-left icon-vertical-align-start elementor-widget elementor-widget-the7_icon_box_grid_widget\" data-id=\"1467c94\" data-element_type=\"widget\" data-widget_type=\"the7_icon_box_grid_widget.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<div class=\"the7-box-grid-wrapper the7-elementor-widget loading-effect-none the7_icon_box_grid_widget-1467c94\">\t\t\t<div class=\"dt-css-grid\">\n\t\t\t\t\t\t\t\t\t<div class=\"wf-cell shown\">\n\t\t\t\t\t\t<div class=\"the7-icon-box-grid\">\t\t\t\t\t\t\t<div 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aria-label=\"Aggravation de l\u2019impact sur la vie quotidienne (par exemple,  incapacit\u00e9 fonctionnelle)&lt;sup&gt;32,33&lt;\/sup&gt;\">\t\t\t\t\t\t\t\t\t\t<div class=\"elementor-icon\">\n\t\t\t\t\t\t\t\t\t\t\t<svg xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"401\" height=\"401\" viewBox=\"0 0 401 401\" fill=\"none\"><g clip-path=\"url(#clip0_313_701)\"><path opacity=\"0.3\" d=\"M373.693 26.3073C338.617 -8.7691 281.786 -8.7691 246.71 26.3073C233.174 39.8429 224.997 56.6064 221.92 74.1016C221.834 74.5104 221.705 74.7902 221.618 75.1775C212.301 123.94 223.039 158.952 161.516 220.497C99.9717 282.021 92.0743 265.623 41.4402 282.537L41.6123 282.731C32.8756 285.571 24.5908 290.22 17.6402 297.149C-5.88006 320.691 -5.88006 358.823 17.6402 382.343C41.1819 405.886 79.3135 405.886 102.834 382.343C109.784 375.393 114.433 367.108 117.273 358.371L117.467 358.543C134.381 307.887 117.983 300.011 179.506 238.466C241.029 176.921 276.061 187.659 324.823 178.362C325.232 178.298 325.491 178.147 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stroke-linejoin=\"round\"><\/path><path d=\"M158.937 371V243.522\" stroke=\"#005EA7\" stroke-width=\"7.58\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><\/path><path d=\"M185.936 371L191.331 290.004\" stroke=\"#005EA7\" stroke-width=\"7.58\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><\/path><path d=\"M223.724 371V246.411\" stroke=\"#005EA7\" stroke-width=\"7.58\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><\/path><path d=\"M196.725 371L191.331 290.004\" stroke=\"#005EA7\" stroke-width=\"7.58\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><\/path><path d=\"M145.43 110H239.102C265.243 110 263.337 74.43 239.102 73.956C247.354 61.4171 234.229 46.5945 220.519 44.3754C199.965 41.036 187.252 61.0724 187.252 61.0724C187.252 61.0724 167.803 47.8226 154.613 53.0794C137.286 59.9952 143.697 77.4462 143.697 77.4462C143.697 77.4462 123.252 73.8052 118.725 88.6278C114.199 103.45 131.807 110 145.386 110H145.43Z\" fill=\"#005EA7\"><\/path><\/g><defs><clipPath id=\"clip0_313_701\"><rect width=\"400.451\" height=\"400.451\" fill=\"white\" transform=\"translate(0.00170898)\"><\/rect><\/clipPath><\/defs><\/svg>\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t<\/a>\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"box-content\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<h5 class=\"box-heading\">\n\t\t\t\t\t\t\t\t\t\t\t<a aria-label=\"Aggravation de l\u2019impact sur la vie quotidienne (par exemple,  incapacit\u00e9 fonctionnelle)&lt;sup&gt;32,33&lt;\/sup&gt;\">\t\t\t\t\t\t\t\t\t\t\t\tAggravation de l\u2019impact sur la vie quotidienne (par exemple,  incapacit\u00e9 fonctionnelle)<sup>32,33<\/sup>\t\t\t\t\t\t\t\t\t\t\t<\/a>\t\t\t\t\t\t\t\t\t\t<\/h5>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/div>\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t<\/div>\n\t\t<\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-d35d89c elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"d35d89c\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<ul><li><strong>La douleur peut \u00eatre un indicateur pronostique de progression<\/strong><sup>34<\/sup> et peut \u00eatre <strong>associ\u00e9e \u00e0 de moins bons r\u00e9sultats<\/strong><sup>35<\/sup>.<\/li><li><strong>Le risque de progression peut \u00eatre plus \u00e9lev\u00e9 pour les plus grosses tumeurs<\/strong><sup>3,33<\/sup>.<\/li><\/ul>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-55db4d2 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"55db4d2\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<p><sup>\u2020<\/sup>&nbsp;Les patients atteints de tumeurs desmo\u00efdes primaires ou r\u00e9cidivantes (n&nbsp;=&nbsp;160) ont \u00e9t\u00e9 identifi\u00e9s r\u00e9trospectivement \u00e0 partir d\u2019une base de donn\u00e9es institutionnelle. Parmi les patients en observation initiale pour lesquels des s\u00e9ries d\u2019examens d\u2019IRM \u00e9taient disponibles, 14&nbsp;sur&nbsp;24 (58&nbsp;%) ayant re\u00e7u un traitement actif n\u2019ont pas pr\u00e9sent\u00e9 de croissance tumorale selon les crit\u00e8res RECIST<sup>31<\/sup>. \u00c9labor\u00e9 \u00e0 partir du texte, r\u00e9f\u00e9rence&nbsp;31.<\/p>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-dd7c57d elementor-widget elementor-widget-the7_button_widget\" data-id=\"dd7c57d\" data-element_type=\"widget\" data-widget_type=\"the7_button_widget.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<div class=\"elementor-button-wrapper\"><a href=\"https:\/\/www.desmoidtumours.eu\/fr\/prise-en-charge\/#disease-progression-identification\" class=\"box-button elementor-button elementor-size-md\">En savoir plus sur les options th\u00e9rapeutiques apr\u00e8s l\u2019identification pr\u00e9coce de la progression d\u2019une tumeur desmo\u00efde<\/a><\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-b5c37e6 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"b5c37e6\" data-element_type=\"widget\" id=\"video-header\" data-widget_type=\"the7-heading.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<h4 class=\"the7-heading-title elementor-size-default\">\u00c9coutez le point de vue d\u2019un oncologue m\u00e9dical sur les tumeurs desmo\u00efdes<\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-15ffe2e e-grid e-con-full e-con e-child\" data-id=\"15ffe2e\" data-element_type=\"container\" id=\"video-grid\">\n\t\t\t\t<div class=\"elementor-element elementor-element-440cc9c elementor-widget elementor-widget-video\" data-id=\"440cc9c\" data-element_type=\"widget\" data-settings=\"{&quot;video_type&quot;:&quot;vimeo&quot;}\" data-widget_type=\"video.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t<div class=\"elementor-wrapper elementor-open-inline\">\n\t\t\t<iframe class=\"elementor-video-iframe\" allowfullscreen allow=\"clipboard-write\" title=\"Lecteur vid\u00e9o vimeo\" src=\"https:\/\/player.vimeo.com\/video\/772212563?color&amp;autopause=0&amp;loop=0&amp;muted=0&amp;title=1&amp;portrait=1&amp;byline=1&amp;h=83264019c6#t=\"><\/iframe>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-e719cb6 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"e719cb6\" data-element_type=\"widget\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<h5>Comprendre les tumeurs desmo\u00efdes<\/h5><p>Regardez la vid\u00e9o du Dr Riedel, oncologue m\u00e9dical au Duke Health et Duke Cancer Institute, dans laquelle il donne une vue d\u2019ensemble sur les tumeurs desmo\u00efdes, y compris en mati\u00e8re de physiopathologie, de pr\u00e9sentations cliniques et d\u2019impact sur les patients.<\/p>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-c49dc9f elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"c49dc9f\" data-element_type=\"widget\" id=\"video-text\" data-widget_type=\"text-editor.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t\t\t<p>APC, g\u00e8ne de l\u2019ad\u00e9nomatose polypose coli&nbsp;; TDM, tomodensitom\u00e9trie&nbsp;; CTNNB1, g\u00e8ne de la cat\u00e9nine \u03b21&nbsp;; PAF, polypose ad\u00e9nomateuse familiale&nbsp;;&nbsp;IRM, imagerie par r\u00e9sonance magn\u00e9tique&nbsp;; NICD, domaine intracellulaire Notch&nbsp;; RECIST, Response Evaluation Criteria In Solid Tumors (crit\u00e8res d\u2019\u00e9valuation de la r\u00e9ponse dans les tumeurs solides)&nbsp;; S45F, type de mutation&nbsp;; T41A, type de mutation&nbsp;; Wnt, site d\u2019int\u00e9gration li\u00e9 \u00e0 Wingless.<\/p>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-01e99e5 e-con-full e-flex e-con e-parent\" data-id=\"01e99e5\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t<div class=\"elementor-element elementor-element-01cd00d e-flex e-con-boxed e-con e-child\" data-id=\"01cd00d\" data-element_type=\"container\">\n\t\t\t\t\t<div class=\"e-con-inner\">\n\t\t<div class=\"elementor-element elementor-element-ab49753 e-con-full e-flex e-con e-child\" data-id=\"ab49753\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-9ecc9fc elementor-widget elementor-widget-the7-accordion\" data-id=\"9ecc9fc\" data-element_type=\"widget\" data-widget_type=\"the7-accordion.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t<div class=\"elementor-accordion the7-adv-accordion\" data-accordion-type=\"accordion\" role=\"tablist\">\n\t\t\t\t\t\t\t<div class=\"elementor-accordion-item\">\n\t\t\t\t\t<h5 id=\"elementor-tab-title-1661\" class=\"elementor-tab-title the7-accordion-header deactive-default\" data-tab=\"1\" role=\"tab\" aria-controls=\"elementor-tab-content-1661\">\n\n\t\t\t\t\t\t\t\t\t\t\t\t\t<span class=\"elementor-accordion-icon elementor-accordion-icon-left\" aria-hidden=\"true\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<span class=\"elementor-accordion-icon-closed\"><svg class=\"e-font-icon-svg e-fas-plus\" viewBox=\"0 0 448 512\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\"><path d=\"M416 208H272V64c0-17.67-14.33-32-32-32h-32c-17.67 0-32 14.33-32 32v144H32c-17.67 0-32 14.33-32 32v32c0 17.67 14.33 32 32 32h144v144c0 17.67 14.33 32 32 32h32c17.67 0 32-14.33 32-32V304h144c17.67 0 32-14.33 32-32v-32c0-17.67-14.33-32-32-32z\"><\/path><\/svg><\/span>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<span class=\"elementor-accordion-icon-opened\"><svg class=\"e-font-icon-svg e-fas-minus\" viewBox=\"0 0 448 512\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\"><path d=\"M416 208H32c-17.67 0-32 14.33-32 32v32c0 17.67 14.33 32 32 32h384c17.67 0 32-14.33 32-32v-32c0-17.67-14.33-32-32-32z\"><\/path><\/svg><\/span>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/span>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<a class=\"elementor-accordion-title\" href=\"\">R\u00e9f\u00e9rences<\/a>\n\t\t\t\t\t<\/h5>\n\t\t\t\t\t<div id=\"elementor-tab-content-1661\" class=\"elementor-tab-content elementor-clearfix deactive-default\" data-tab=\"1\" role=\"tabpanel\" aria-labelledby=\"elementor-tab-title-1661\"><ol>\n<li>Kasper B et al. Desmoid Working Group. An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma Patients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)\/Soft Tissue and Bone Sarcoma Group (STBSG). <em>Ann Oncol<\/em>. 2017;28(10):2399-2408.<\/li>\n<li>Penel N et al. Adult desmoid tumors: biology, management and ongoing trials. <em>Curr Opin Oncol<\/em>. 2017;29(4):268-274.<\/li>\n<li>Gronchi A et al. Desmoid tumor working group. The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients. <em>Eur J Cancer<\/em>. 2020;127:96-107.<\/li>\n<li>Bonvalot S et al. The treatment of desmoid tumors: a stepwise clinical approach.<em> Ann Oncol<\/em>. 2012;23(suppl 10):x158-x166.<\/li>\n<li>Constantinidou A et al. Clinical presentation of desmoid tumors. In: Litchman C, ed. Desmoid Tumors. Springer; 2012:chap 2. Consult\u00e9 le 9&nbsp;avril&nbsp;2024. <a href=\"https:\/\/www.researchgate.net\/publication\/226455135\" target=\"_blank\" rel=\"noopener\">https:\/\/www.researchgate.net\/publication\/226455135<\/a>.<\/li>\n<li>Joglekar SB et al. Current perspectives on desmoid tumors: the mayo clinic approach. <em>Cancers<\/em> (<em>Basel<\/em>). 2011;3(3):3143-3155.<\/li>\n<li>Penel N et al. Surgical versus non-surgical approach in primary desmoid-type fibromatosis patients: a nationwide prospective cohort from the French Sarcoma Group. <em>Eur J Cancer<\/em>. 2017;83:125-131.<\/li>\n<li>Shinagare AB et al. A to Z of desmoid tumors. <em>AJR Am J Roentgenol<\/em>. 2011;197(6):W1008-W1014.<\/li>\n<li>Koshariya M et al. Giant desmoid tumor of the anterior abdominal wall in a young female: a case report. <em>Case Rep Surg<\/em>. 2013;2013:780862.<\/li>\n<li>McDonald ES et al. Best cases from the AFIP: extraabdominal desmoid-type fibromatosis. <em>Radiographics<\/em>. 2008;28(3):901-906.<\/li>\n<li>Abr\u00e3o FC et al. Desmoid tumors of the chest wall: surgical challenges and possible risk factors. <em>Clinics<\/em> (<em>Sao Paulo<\/em>). 2011;66(4):705-708.<\/li>\n<li>Xie Y et al. Recurrent desmoid tumor of the mediastinum: a case report. <em>Oncol Lett<\/em>. 2014;8(5):2276-2278.<\/li>\n<li>Scaramussa FS et al. Desmoid tumor in hand: a case report. <em>SM J Orthop<\/em>. 2016;2(3):1036.<\/li>\n<li>Baranov E et al. Soft tissue special issue: fibroblastic and myofibroblastic neoplasms of the head and neck. <em>Head Neck Pathol<\/em>. 2020;14(1):43-58.<\/li>\n<li>Ingley KM et al. High prevalence of persistent emotional distress in desmoid tumor. <em>Psychooncology<\/em>. 2020;29(2):311-320.<\/li>\n<li>Gounder MM et al. Prospective development of a patient-reported outcomes instrument for desmoid tumors or aggressive fibromatosis. <em>Cancer<\/em>. 2020;126(3):531-539.<\/li>\n<li>Bektas M et al. Desmoid tumors: a comprehensive review. <em>Adv Ther<\/em>. 2023;40(9):3697-3722.<\/li>\n<li>Kasper B et al. Desmoid Tumor Working Group. Current management of desmoid tumors: a review. <em>JAMA Oncol<\/em>. 2024;10(8):1121-1128.<\/li>\n<li>Ravi V et al. Desmoid tumors: epidemiology, molecular pathogenesis, clinical presentation, diagnosis, and local therapy Jun 2024 UptoDate.<\/li>\n<li>Skubitz KM. Biology and treatment of aggressive fibromatosis or desmoid tumor. <em>Mayo Clin Proc<\/em>. 2017;92(6):947-964.<\/li>\n<li>Lopez R et al. Problems in diagnosis and management of desmoid tumors. <em>Am J Surg<\/em>. 1990;159(5):450-453.<\/li>\n<li>Kasper B et al. Desmoid tumors: clinical features and treatment options for advanced disease. <em>Oncologist<\/em>. 2011;16(5):682-93.<\/li>\n<li>Crago AM et al. Near universal detection of alterations in CTNNB1 and Wnt pathway regulators in desmoid-type fi bromatosis by whole-exome sequencing and genomic analysis. <em>Genes Chromosomes Cancer<\/em>. 2015;54(10):606-615.<\/li>\n<li>Gronchi A et al. Desmoid tumor working group. The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients [supplementary appendix]. <em>Eur J Cancer<\/em>. 2020;127:96-107.<\/li>\n<li>Shang H et al. Targeting the Notch pathway: a potential therapeutic approach for desmoid tumors. <em>Cancer<\/em>. 2015;121(22):4088-4096.<\/li>\n<li>Wang YF et al. Postoperative recurrence of desmoid tumors: clinical and pathological perspectives. <em>World J Surg Oncol<\/em>. 2015;13:26.<\/li>\n<li>Easter DW et al. Recent trends in the management of desmoid tumors. Summary of 19 cases and review of the literature. <em>Ann Surg<\/em>. 1989;210(6):765-769.<\/li>\n<li>Napolitano A et al. Recent advances in desmoid tumor therapy. <em>Cancers<\/em> (<em>Basel<\/em>). 2020;12(8):2135.<\/li>\n<li>Lazar AJ et al. Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors. <em>Am J Pathol<\/em>. 2008;173(5):1518-27.<\/li>\n<li>Timbergen MJM et al. The prognostic role of \u03b2-Catenin mutations in desmoid-type fibromatosis undergoing resection only: a meta-analysis of individual patient data. <em>Ann Surg<\/em>. 2021;273(6):1094-1101.<\/li>\n<li>Cassidy MR et al. Association of MRI T2 signal intensity with desmoid tumor progression during active observation: a retrospective cohort study. <em>Ann Surg<\/em>. 2020;271(4):748-755.<\/li>\n<li>R\u00e9f\u00e9renc\u00e9 avec l\u2019autorisation des lignes directrices en mati\u00e8re de pratique clinique en oncologie du NCCN (lignes directrices du NCCN\u00ae) pour le sarcome des tissus mous V.1.2024. \u00a9 National Comprehensive Cancer Network, Inc. 2024. Tous droits r\u00e9serv\u00e9s. Consult\u00e9 le 29&nbsp;avril&nbsp;2024. Pour consulter la derni\u00e8re version compl\u00e8te des lignes directrices, rendez-vous en ligne sur NCCN.org. Le NCCN n\u2019offre aucune garantie de quelque sorte, que ce soit concernant leur contenu, leur utilisation ou leur application et d\u00e9cline toute responsabilit\u00e9 quant \u00e0 leur quelconque application ou utilisation.<\/li>\n<li>Kasper B et al. Desmoid Tumor Working Group. Current management of desmoid tumors: a review. [supplementary appendix] <em>JAMA Oncol<\/em>. 2024;10(8):1121-1128.<\/li>\n<li>Cuomo P et al. Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study. <em>BMC Cancer<\/em>. 2021;21(1):437.<\/li>\n<li>Penel N et al. Pain in desmoid-type fibromatosis: prevalence, determinants and prognosis value.<em> Int J Cancer<\/em>. 2023;153(2):407-416.<\/li>\n<\/ol>\n<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t","protected":false},"excerpt":{"rendered":"<p>Vue d\u2019ensemble de la maladie \u00c9tiologie et pathogen\u00e8se Principes de prise en charge \u00c0 propos des tumeurs desmo\u00efdes Vue d\u2019ensemble de la maladie Une maladie infiltrante et agressive1 Les tumeurs desmo\u00efdes sont des tumeurs localement agressives des tissus mous potentiellement morbides, qui ont tendance \u00e0 infiltrer les structures environnantes et \u00e0 se propager le long&hellip;<\/p>\n","protected":false},"author":4,"featured_media":0,"parent":0,"menu_order":5,"comment_status":"closed","ping_status":"closed","template":"elementor_header_footer","meta":{"_acf_changed":false,"footnotes":""},"class_list":["post-21281","page","type-page","status-publish","hentry"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v24.6 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Tumeurs desmo\u00efdes\u00a0: \u00e0 propos des tumeurs desmo\u00efdes | SpringWorks Therapeutics<\/title>\n<meta name=\"description\" content=\"Vue d&#039;ensemble de la maladie\u00a0: d\u00e9couvrez l\u2019\u00e9tiologie, la pathogen\u00e8se et les principes de prise en charge des tumeurs desmo\u00efdes.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.desmoidtumours.eu\/fr\/a-propos-des-tumeurs-desmoides\/\" \/>\n<meta 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