{"id":21936,"date":"2024-08-07T10:14:05","date_gmt":"2024-08-07T10:14:05","guid":{"rendered":"https:\/\/www.desmoidtumours.eu\/informazioni-sui-tumori-desmoidi\/"},"modified":"2026-02-10T10:33:58","modified_gmt":"2026-02-10T10:33:58","slug":"informazioni-sui-tumori-desmoidi","status":"publish","type":"page","link":"https:\/\/www.desmoidtumours.eu\/it\/informazioni-sui-tumori-desmoidi\/","title":{"rendered":"Informazioni sui tumori desmoidi"},"content":{"rendered":"\t\t<div data-elementor-type=\"wp-page\" data-elementor-id=\"21936\" class=\"elementor elementor-21936 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\">Panoramica della malattia<\/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\">Eziologia e patogenesi<\/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\">Principi di gestione<\/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\">Informazioni sui tumori desmoidi<\/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\">Panoramica della malattia<\/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\">Una malattia infiltrante e aggressiva<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>\n \t<li>I tumori desmoidi sono tumori <strong>localmente aggressivi<\/strong> dei tessuti molli, potenzialmente associati a morbilit\u00e0, con tendenza a infiltrare le strutture circostanti e a diffondersi lungo le fasce e i muscoli<sup>1,2<\/sup><\/li>\n \t<li>A volte indicati come fibromatosi aggressiva o fibromatosi desmoide,<sup>3<\/sup> questi tumori mesenchimali possono essere <strong>gravi<\/strong>, <strong>debilitanti<\/strong> e, in rari casi, causare persino danni <strong>potenzialmente letali<\/strong> agli organi <sup>2<\/sup><\/li>\n \t<li>I tumori desmoidi possono <strong>infiltrare i tessuti adiacenti comprimendo muscoli, nervi e vasi<\/strong><sup>4.5<\/sup><\/li>\n<\/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\">Sintomatologia e sedi tumorali<\/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\">I tumori desmoidi possono causare morbilit\u00e0 gravi e debilitanti<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>\n \t<li>La sintomatologia correlata ai tumori desmoidi varia in base alla sede dell\u2019insorgenza tumorale<sup>5.6<\/sup><\/li>\n \t<li>Uno studio di coorte prospettico ha evidenziato che i tumori della <strong>parete toracica<\/strong>, <strong>degli arti superiori<\/strong> <strong>e della testa e del collo<\/strong> sono associati a esiti sfavorevoli dopo l\u2019intervento chirurgico<sup>7<\/sup><\/li>\n<\/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=\"\/it\/esperienza-del-della-paziente\/#disease-burden\" class=\"box-button elementor-button elementor-size-md\">Maggiori informazioni sulla gestione dei tumori desmoidi<\/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\n<table id=\"tablepress-7\" class=\"tablepress tablepress-id-7 first-tumors-table\">\n<thead>\n<tr class=\"row-1\">\n\t<th class=\"column-1\">SEDE DEL TUMORE<\/th><th class=\"column-2\">FREQUENZA STIMATA<sup>5<\/sup>\u200b<\/th><th class=\"column-3\">SINTOMI E COMPLICANZE COMUNI<\/th>\n<\/tr>\n<\/thead>\n<tbody class=\"row-striping\">\n<tr class=\"row-2\">\n\t<td class=\"column-1\"><strong>Intra-addominale<\/strong><br \/><small>(incluso il mesentere)<\/small><\/td><td class=\"column-2\"><strong>20%<\/strong><\/td><td class=\"column-3\">La compressione pu\u00f2 causare dolore, cachessia, malessere, distensione addominale o ostruzione dell\u2019intestino o degli ureteri<sup>5,8<\/sup><\/td>\n<\/tr>\n<tr class=\"row-3\">\n\t<td class=\"column-1\"><strong>Parete addominale<\/strong><\/td><td class=\"column-2\"><strong>16%<\/strong><\/td><td class=\"column-3\">I tumori di grandi dimensioni possono causare stiramento dei tessuti, compressione dei vasi sanguigni e spostamento dell\u2019intestino o della vescica<sup>9<\/sup><\/td>\n<\/tr>\n<tr class=\"row-4\">\n\t<td class=\"column-1\"><strong>Arti inferiori<\/strong><\/td><td class=\"column-2\"><strong>16%<\/strong><\/td><td class=\"column-3\">Mobilit\u00e0 limitata, dolore, rigidit\u00e0 muscolare o deformit\u00e0<sup>5,10<\/sup><\/td>\n<\/tr>\n<tr class=\"row-5\">\n\t<td class=\"column-1\"><strong>Parete toracica<\/strong><\/td><td class=\"column-2\"><strong>15%<\/strong><\/td><td class=\"column-3\">Dispnea,<sup>5<\/sup> disfagia,<sup>5<\/sup> invasione pleurica,<sup>8<\/sup> coinvolgimento della colonna vertebrale<sup>11<\/sup>  o delle coste,<sup>12<\/sup> erosione ossea,<sup>12<\/sup> e dolore<sup>12<\/sup><\/td>\n<\/tr>\n<tr class=\"row-6\">\n\t<td class=\"column-1\"><strong>Arti superiori<\/strong><\/td><td class=\"column-2\"><strong>14%<\/strong><\/td><td class=\"column-3\">Mobilit\u00e0 ridotta, coinvolgimento di muscoli e legamenti, debolezza dell\u2019arto, deformit\u00e0 o dolore<sup>5,13<\/sup><\/td>\n<\/tr>\n<tr class=\"row-7\">\n\t<td class=\"column-1\"><strong>Testa e Collo<\/strong><\/td><td class=\"column-2\"><strong>8%<\/strong><\/td><td class=\"column-3\">Dolore, deficit neurologici, prossimit\u00e0 a strutture vitali, incluso il rischio di morte per compromissione vascolare o delle vie aeree<sup>14<\/sup><\/td>\n<\/tr>\n<tr class=\"row-8\">\n\t<td class=\"column-1\"><strong>Altro<\/strong><\/td><td class=\"column-2\"><strong>11%<\/strong><\/td><td class=\"column-3\">Sintomi e complicanze dipendenti dalla sede<sup>5,6<\/sup><\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<!-- #tablepress-7 from cache -->\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 posizione dei tumori desmoidi pu\u00f2 influire significativamente sulla qualit\u00e0 della vita.<\/strong><sup>15<\/sup> Per esempio, un tumore desmoide con neoformazioni \u201ca viticcio\u201d<sup>1<\/sup>, che avvolgono <strong>i nervi<\/strong>, pu\u00f2 essere associato a <strong>dolore neuropatico debilitante<\/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=\"\/it\/esperienza-del-della-paziente\/#disease-burden\" class=\"box-button elementor-button elementor-size-md\">Maggiori informazioni sul carico di malattia dei tumori desmoidi<\/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\">Decorso della malattia<\/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\">Variabile e imprevedibile<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>I tumori desmoidi sono caratterizzati da un decorso della malattia variabile e imprevedibile a seconda della sede del tumore e della morbilit\u00e0 associata<sup>1.5<\/sup><\/li><li>Circa il 50% dei tumori desmoidi mostra una biologia aggressiva continuando a crescere o diventando sintomatico. La grande maggioranza delle progressioni (89%) si verifica entro i primi 2 anni di osservazione<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=\"800\" height=\"451\" src=\"data:image\/svg+xml,%3Csvg%20xmlns%3D&#039;http%3A%2F%2Fwww.w3.org%2F2000%2Fsvg&#039;%20viewBox%3D&#039;0%200%20800%20451&#039;%2F%3E\" class=\"attachment-full size-full preload-me aspect lazy lazy-load\" alt=\"\" sizes=\"(max-width: 800px) 100vw, 800px\" style=\"--ratio: 800 \/ 451;\" data-src=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/02\/Desmoid-Tumours-Clinical.jpg\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/02\/Desmoid-Tumours-Clinical.jpg 800w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/02\/Desmoid-Tumours-Clinical-300x169.jpg 300w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/02\/Desmoid-Tumours-Clinical-768x433.jpg 768w\" \/><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 >Decorso clinico dei tumori desmoidi<\/a><\/h6><div class=\"box-description\">Elaborato dal testo, riferimento 17.<br \/>\n*Il 10-28% dei tumori desmoidi si risolver\u00e0\nspontaneamente senza trattamento<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\">Incidenza e fattori di rischio<\/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=\"Epidemiologia\">\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 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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=\"Epidemiologia\">\t\t\t\t\t\t\t\t\t\t\t\tEpidemiologia\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>Il tumore desmoide ha un\u2019incidenza di circa <strong>5 casi per 1 milione di popolazione<\/strong> all\u2019anno.<sup>18<\/sup><\/p>\n<p>L'Orphanet Report Series indica un\u2019incidenza annuale media di 3 casi di tumori desmoidi per milione di anni-persona in Europa.<sup>17<\/sup><\/p>\n<p>Gli studi epidemiologici rivelano che la maggior parte dei casi di tumore desmoide compare nella <strong>fascia di et\u00e0 compresa tra i 20 e i 44 anni<\/strong>.<sup>17<\/sup><\/p>\n<ul>\n \t<li>I tumori desmoidi sono rari nei giovani e negli anziani<sup>19<\/sup><\/li>\n<li>La stima dell\u2019incidenza \u00e8 aumentata negli anni pi\u00f9 recenti, il che potrebbe essere correlato a un miglioramento delle tecniche diagnostiche<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=\"Sesso femminile\">\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 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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=\"Sesso femminile\">\t\t\t\t\t\t\t\t\t\t\t\tSesso femminile\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>Il rapporto donne-uomini \u00e8 di circa 2-3:1,<sup>20<\/sup><\/p>\n<ul>\n<li>La gravidanza pu\u00f2 favorire lo sviluppo di tumori desmoidi<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=\"Trauma precedente\">\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 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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=\"Trauma precedente\">\t\t\t\t\t\t\t\t\t\t\t\tTrauma precedente\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>Circa il 25%<\/strong> dei pazienti con tumori desmoidi presenta un\u2019anamnesi di precedente trauma.<sup>5<\/sup>\n<\/p>\n<ul>\n<li>Lesioni o interventi chirurgici possono aumentarne il rischio<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=\"Mutazioni del gene &lt;i&gt;APC&lt;\/i&gt;(adenomatous polyposis coli, gene della poliposi adenomatosa del colon)\">\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\" 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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=\"Mutazioni del gene &lt;i&gt;APC&lt;\/i&gt;(adenomatous polyposis coli, gene della poliposi adenomatosa del colon)\">\t\t\t\t\t\t\t\t\t\t\t\tMutazioni del gene <i>APC<\/i>(adenomatous polyposis coli, gene della poliposi adenomatosa del colon)\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>Le <strong>caratteristiche fenotipiche<\/strong> sono correlate alla posizione della mutazione APC rispetto a determinati codoni.<sup>22<\/sup>\n<\/p>\n<ul>\n \t<li>I pazienti con poliposi adenomatosa familiare (familial adenomatous polyposis, FAP) presentano un <strong>rischio 1.000 volte maggiore<\/strong> di sviluppare tumori desmoidi rispetto alla popolazione generale<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\">Eziologia e patogenesi dei tumori desmoidi <\/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 comprensione della patogenesi molecolare \u00e8 di notevole interesse in questa malattia a causa dell'elevata variabilit\u00e0 del suo decorso clinico<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\u2019eziologia dei tumori desmoidi non \u00e8 nota. Tuttavia, l\u2019identificazione di alterazioni cromosomiche clonali in una percentuale significativa di casi supporta la natura neoplastica di questi tumori.<sup>19<\/sup><\/p>\n<ul>\n<li>Si ritiene che la patogenesi dei tumori desmoidi sia collegata a traumi\n<ul>\n<li>l&#8217;intervento chirurgico pu\u00f2&nbsp;talvolta favorire la crescita del tumore.<sup>4<\/sup><\/li>\n<li>I fattori di crescita rilasciati dopo l\u2019intervento chirurgico, durante la fase iniziale della guarigione della ferita, potrebbero trasmettere segnali che promuovono l\u2019attivazione della \u03b2-catenina<sup>4<\/sup><\/li>\n<\/ul>\n<\/li>\n<li>La gravidanza pu\u00f2 promuovere il tumore desmoide non solo a causa di effetti ormonali, ma anche a causa del rilascio sistemico di fattori di crescita<sup>4<\/sup><\/li>\n<li>Sono stati implicati due pathway nel <strong>pathway canonico Wnt\/<b>\u03b2<\/b>-catenina\/APC<\/strong>, in cui le mutazioni del gene <i>CTNNB1<\/i> o del gene <i>APC<\/i> portano all\u2019accumulo di \u03b2-catenina e nel <strong>pathway Notch<\/strong>, in cui si verificano due scissioni dei recettori Notch <sup>17<\/sup>\n<ul>\n<li>Si ritiene che nella patogenesi dei tumori desmoidi siano coinvolte la potenziale interferenza tra il pathway Notch e Wnt, cos\u00ec come l\u2019attivazione del pathway Notch derivante dalla disregolazione del pathway Wnt.<sup>17<\/sup><\/li>\n<\/ul>\n<\/li>\n<\/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 >Accumulo nucleare di \u03b2-catenina<\/a><\/h6><div class=\"box-description\"><ul>\n \t<li>La disregolazione del pathway Wnt\/\u03b2-catenina \u00e8 stata implicata nella patogenesi dei tumori desmoidi<sup>2,23<\/sup><\/li>\n \t<li>L\u2019accumulo nucleare di \u03b2-catenina e la \ntrascrizione del gene a valle sono fattori chiave della proliferazione delle cellule tumorali desmoidi<sup>2,24<\/sup><\/li>\n \t<li>L\u2019accumulo nucleare di \u03b2-catenina pu\u00f2 essere avviato da<sup>2,24<\/sup>\n<ul>\n \t<li>Mutazioni attivanti del gene della \u03b2-catenina <em>CTNNB1<\/em><\/li>\n \t<li>Mutazioni inattivanti del gene onco-soppressore <em>APC<\/em>, spesso riscontrate nella poliposi adenomatosa familiare (FAP)<\/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 >Attivazione di Notch da parte della \u03b3-secretasi<\/a><\/h6><div class=\"box-description\"><ul>\n \t<li>Il pathway di Notch pu\u00f2 essere attivo nei tumori desmoidi<sup>25<\/sup><\/li>\n \t<li>Quando disregolato, Notch pu\u00f2 attivare una trasmissione di segnali che contribuiscono alla crescita tumorale<sup>25<\/sup><\/li>\n \t<li>La trasmissione dei segnali del recettore di Notch richiede l\u2019attivazione proteolitica da parte dell\u2019enzima \u03b3-secretasi (gamma secretasi)<sup>25<\/sup><\/li>\n \t<li>Il clivaggio della \u03b3-secretasi rilascia il dominio intracellulare Notch (Notch intracellular domain, NICD) che trasloca nel nucleo e attiva la trascrizione genica<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 >Interazione dei pathway<\/a><\/h6><div class=\"box-description\"><ul>\n \t<li>L\u2019interazione tra il pathway di Wnt\/\u03b2-catenina e di Notch pu\u00f2 contribuire ulteriormente alla patogenesi del tumore desmoide<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<div class=\"elementor-element elementor-element-ebec44e e-con-full elementor-hidden-desktop elementor-hidden-laptop elementor-hidden-tablet_extra elementor-hidden-tablet elementor-hidden-mobile e-flex e-con e-child\" data-id=\"ebec44e\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-6ba77ba e-con-full e-flex e-con e-child\" data-id=\"6ba77ba\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-f91478c content-align-center icon-vertical-align-center no-link icon-box-change-position-30 bord-img-8 icon-box-vertical-align-top elementor-widget elementor-widget-the7_image_box_widget\" data-id=\"f91478c\" 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-f91478c\">\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 >Accumulo nucleare di \u03b2-catenina<\/a><\/h6>\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<div class=\"elementor-element elementor-element-004e882 e-con-full e-flex e-con e-child\" data-id=\"004e882\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-3a92178 elementor-widget-tablet__width-inherit bullet-custom  elementor-widget elementor-widget-text-editor\" data-id=\"3a92178\" 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>\n \t<li>La disregolazione del pathway Wnt\/\u03b2-catenina \u00e8 stata implicata nella patogenesi dei tumori desmoidi<sup>2,24<\/sup><\/li>\n \t<li>L\u2019accumulo nucleare di \u03b2-catenina e la \ntrascrizione del gene a valle sono fattori chiave della proliferazione delle cellule tumorali desmoidi<sup>2,25<\/sup><\/li>\n \t<li>L\u2019accumulo nucleare di \u03b2-catenina pu\u00f2 essere avviato da<sup>2,25<\/sup>\n<ul>\n \t<li>Mutazioni attivanti del gene della \u03b2-catenina <em>CTNNB1<\/em><\/li>\n \t<li>Mutazioni inattivanti nel regolatore negativo <em>APC<\/em>, spesso nella poliposi adenomatosa familiare (FAP)<\/li>\n<\/ul>\n<\/li>\n<\/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>\n\t\t<div class=\"elementor-element elementor-element-dd077d8 e-con-full elementor-hidden-desktop elementor-hidden-laptop elementor-hidden-tablet_extra elementor-hidden-tablet elementor-hidden-mobile e-flex e-con e-child\" data-id=\"dd077d8\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-9c6cb26 e-con-full e-flex e-con e-child\" data-id=\"9c6cb26\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-ad873bd content-align-center icon-vertical-align-center no-link icon-box-change-position-30 bord-img-8 icon-box-vertical-align-top elementor-widget elementor-widget-the7_image_box_widget\" data-id=\"ad873bd\" 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-ad873bd\">\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.png\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Notch-activation-by-gamma-secretase.png 512w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Notch-activation-by-gamma-secretase-221x300.png 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 >Attivazione di Notch da parte della \u03b3-secretasi<\/a><\/h6>\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<div class=\"elementor-element elementor-element-3c9bb61 e-con-full e-flex e-con e-child\" data-id=\"3c9bb61\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-f0a110c elementor-widget-tablet__width-inherit bullet-custom  elementor-widget elementor-widget-text-editor\" data-id=\"f0a110c\" 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>\n \t<li>Il pathway di Notch pu\u00f2 essere attivo nei tumori desmoidi<sup>26<\/sup><\/li>\n \t<li>Quando disregolato, Notch pu\u00f2 attivare vie che contribuiscono alla crescita tumorale<sup>26<\/sup><\/li>\n \t<li>La segnalazione del recettore Notch richiede l\u2019attivazione proteolitica da parte dell\u2019enzima <span class=\"ui-provider a b c d e f g h i j k l m n o p q r s t u v w x y z ab ac ae af ag ah ai aj ak\" dir=\"ltr\">\u03b3<\/span>-secretasi<sup>26<\/sup><\/li>\n \t<li>Il clivaggio della <span class=\"ui-provider a b c d e f g h i j k l m n o p q r s t u v w x y z ab ac ae af ag ah ai aj ak\" dir=\"ltr\">\u03b3<\/span>-secretasi rilascia l\u2019NICD che si trasloca nel nucleo e attiva la trascrizione genica<sup>26<\/sup><\/li>\n<\/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>\n\t\t<div class=\"elementor-element elementor-element-252c163 e-con-full elementor-hidden-desktop elementor-hidden-laptop elementor-hidden-tablet_extra elementor-hidden-tablet elementor-hidden-mobile e-flex e-con e-child\" data-id=\"252c163\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-1aeb511 e-con-full e-flex e-con e-child\" data-id=\"1aeb511\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-1688181 content-align-center icon-vertical-align-center no-link icon-box-change-position-30 bord-img-8 icon-box-vertical-align-top elementor-widget elementor-widget-the7_image_box_widget\" data-id=\"1688181\" 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-1688181\">\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.png\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Patchway-crosstalk.png 508w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2024\/08\/Patchway-crosstalk-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 >Interazione dei pathway<\/a><\/h6>\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<div class=\"elementor-element elementor-element-4bdb28f e-con-full e-flex e-con e-child\" data-id=\"4bdb28f\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-789ac44 elementor-widget-tablet__width-inherit bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"789ac44\" 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>\n \t<li>L\u2019interazione tra il pathway di Wnt\/\u03b2-catenina e di Notch pu\u00f2 contribuire ulteriormente alla patogenesi del tumore desmoide<sup>18<\/sup><\/li>\n<\/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>\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=\"Recidiva\"><\/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\">Rischio di recidiva<\/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>I tassi di recidiva possono essere influenzati dalla <strong>sede del tumore<\/strong>:<sup>17<\/sup><ul><li>Si ritiene che i tumori localizzati negli arti siano localmente aggressivi e presentino tassi di recidiva compresi tra il 24% e il 77%<\/li><li>I tassi di recidiva locale dei tumori intra-addominali nei pazienti con FAP sono superiori a quelli dei tumori extra-addominali e sono compresi tra il 57% e l\u201986%<\/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\">Esiste un alto rischio di recidiva locale dopo l\u2019intervento chirurgico<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>I tassi di recidiva possono essere esacerbati da <strong>un trauma<\/strong>, come il trauma <strong>chirurgico<\/strong>, e variano da circa il 25% al 60% a 5 anni<sup>17<\/sup><ul><li>L\u2019invasione dei principali vasi e nervi e la qualit\u00e0 dei margini chirurgici sono fattori chiave per l\u2019elevato tasso di recidiva postoperatoria<sup>26<\/sup><\/li><li>Purtroppo, anche quando i margini chirurgici sono privi di tumore, i <strong>tassi di recidiva sono elevati<\/strong><sup>27<\/sup><\/li><\/ul><\/li><\/ul><p>I fattori di crescita rilasciati dopo l\u2019intervento chirurgico durante la fase iniziale di guarigione della ferita potrebbero promuovere l\u2019attivazione della \u03b2-catenina.<sup>4<\/sup><\/p><ul><li>Il fatto che alcuni tumori siano recidivati dopo l\u2019intervento chirurgico, ma che in seguito siano rimasti stabili senza trattamento, suggerisce che i fattori di crescita rilasciati dopo l\u2019intervento chirurgico potrebbero aver favorito la recidiva nei tumori che altrimenti sarebbero stati indolenti<sup>4<\/sup><\/li><li>La mutazione specifica di CTNNB1 S45F sembra essere associata a una peggiore sopravvivenza libera da recidiva dopo l\u2019intervento chirurgico<sup>28<\/sup><ul><li>La sopravvivenza libera da recidiva a 5 anni era significativamente pi\u00f9 scarsa nei pazienti con tumori desmoidi S45F-mutati (P&lt;0,0001) rispetto ai pazienti con mutazione T41A o con tumori non mutati<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-8\" class=\"tablepress tablepress-id-8 tumors-table\">\n<thead>\n<tr class=\"row-1\">\n\t<th class=\"column-1\">MUTAZIONE<\/th><th class=\"column-2\">SOPRAVVIVENZA LIBERA DA RECIDIVA STIMATA (IC 95%)(95% CI)<\/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 mutato<\/td><td class=\"column-2\">65% (38%-83%)<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<!-- #tablepress-8 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\/2026\/01\/Patients-with-CTNNB1-mutations_IT-1024x577.png\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/01\/Patients-with-CTNNB1-mutations_IT-1024x577.png 1024w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/01\/Patients-with-CTNNB1-mutations_IT-300x169.png 300w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/01\/Patients-with-CTNNB1-mutations_IT-768x433.png 768w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/01\/Patients-with-CTNNB1-mutations_IT-1536x866.png 1536w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/01\/Patients-with-CTNNB1-mutations_IT-2048x1154.png 2048w\" \/><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 >I pazienti con mutazioni di CTNNB1 a livello del codone 45 (45F) presentavano un rischio 3 volte maggiore di recidiva dopo l\u2019intervento chirurgico<sup>29<\/sup><\/a><\/h6><div class=\"box-description\">Elaborato dalla figura 2b, riferimento 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>In una metanalisi \u00e8 stato riportato che le dimensioni del tumore sono un importante mediatore per un aumento del rischio di recidiva nei pazienti con mutazione <em>CTNNB1<\/em> S45F.<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=\"\/it\/gestione\/#surgery\" class=\"box-button elementor-button elementor-size-md\">Scopri di pi\u00f9 sul trattamento chirurgico<\/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\">Principi di gestione<\/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\">\u00c8 fondamentale scegliere attentamente la strategia di gestione per ciascun paziente affetto da tumori desmoidi per ottimizzare il controllo del tumore e migliorare la qualit\u00e0 della vita<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>Nell\u2019ultimo decennio sono stati compiuti importanti sforzi per standardizzare la gestione di questa malattia.<sup>18<\/sup><\/p><p>Attualmente, le esigenze insoddisfatte nell\u2019ambito dei tumori desmoidi includono una diagnosi precoce e accurata e trattamenti approvati indicati per i\/le pazienti con tumori desmoidi.<sup>17<\/sup><\/p><p>In uno studio condotto presso il Memorial Sloan Kettering Cancer Center, il 58% dei pazienti con tumori desmoidi \u00e8 passato dalla sorveglianza attiva al trattamento di prima linea senza progressione radiografica del tumore.<sup>31\u2020<\/sup><\/p><p>Gli obiettivi del trattamento non devono concentrarsi esclusivamente sui marcatori clinici, come la sopravvivenza libera da progressione, ma devono anche considerare gli endpoint rilevanti per il paziente, come una riduzione del carico dei sintomi specifici del tumore desmoide (per es., dolore) e il suo impatto sulla vita dei pazienti, il miglioramento delle attivit\u00e0 quotidiane e la qualit\u00e0 della vita complessiva.<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\">Identificazione precoce della progressione<\/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\u2019identificazione della progressione del tumore desmoide pu\u00f2 portare a un avvio tempestivo del trattamento appropriato <\/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>Per aiutare a migliorare gli esiti dei pazienti, gli operatori sanitari devono <strong>valutare la progressione il prima possibile<\/strong> con almeno uno dei seguenti metodi:<\/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 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=\"Crescita tumorale documentata mediante RM o TC&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_624)\"><path opacity=\"0.3\" d=\"M373.883 26.3094C338.79 -8.78381 281.93 -8.78381 246.837 26.3094C233.295 39.8516 225.114 56.6231 222.035 74.1267C221.949 74.5358 221.82 74.8156 221.733 75.2032C212.411 123.989 223.154 159.018 161.601 220.593C100.027 282.146 92.1252 265.74 41.466 282.662L41.6382 282.856C32.8972 285.698 24.6083 290.349 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TC<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<div class=\"box-description\"><strong>OPPURE<\/strong><\/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=\"Peggioramento dei sintomi&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_647)\"><g opacity=\"0.3\"><path d=\"M375.552 26.4265C340.302 -8.82333 283.188 -8.82333 247.939 26.4265C234.336 40.0291 226.118 56.8755 223.026 74.4572C222.939 74.8681 222.809 75.1492 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class=\"box-description\"><strong>OPPURE<\/strong><\/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=\"Peggioramento dell\u2019impatto sulla vita quotidiana (per es., compromissione funzionale)&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 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funzionale)<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>Il dolore pu\u00f2 essere un indicatore prognostico di progressione<\/strong><sup>34<\/sup> e pu\u00f2 essere <strong>associato a esiti peggiori<\/strong><sup>35<\/sup><\/li><li><strong>Il rischio di progressione pu\u00f2 essere maggiore per i tumori pi\u00f9 grandi<\/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>I pazienti con tumori desmoidi primari o ricorrenti (n=160) sono stati identificati retrospettivamente da una banca dati istituzionale. Tra i pazienti sottoposti a osservazione iniziale per i quali erano disponibili RM seriali, 14 su 24 (58%) sottoposti a trattamento attivo non hanno manifestato crescita tumorale secondo i criteri RECIST.<sup>31<\/sup> Elaborato dal testo, riferimento 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=\"\/it\/gestione\/#disease-progression-identification\" class=\"box-button elementor-button elementor-size-md\">Ottieni informazioni sulle opzioni di trattamento dopo l\u2019identificazione precoce della progressione del tumore desmoide<\/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\">Ascolta i punti di vista di un oncologo medico sui tumori desmoidi<\/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=\"vimeo Video Player\" 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>Comprendere i tumori desmoidi<\/h5><p>Osservare il Dott. Riedel, medico oncologo del Duke Health and Duke Cancer Institute, mentre fornisce una panoramica dei tumori desmoidi che include fisiopatologia, presentazioni cliniche e impatto sui\/sulle pazienti.<\/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, gene della poliposi da adenomatosi del colon; TC, tomografia computerizzata; CTNNB1, gene della catenina \u03b21; FAP, poliposi adenomatosa familiare; RM, risonanza magnetica per immagini; NICD, Notch IntraCellular Domain; RECIST, Criteri di valutazione della risposta nei tumori solidi; S45F, tipo di mutazione; T41A, tipo di mutazione; Wnt, sito di integrazione correlato a 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=\"\">Riferimenti<\/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. Accesso 9 aprile 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.\u00a0<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>Citato con l\u2019autorizzazione delle NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines\u00ae) per il sarcoma dei tessuti molli V.1.2024. \u00a9 National Comprehensive Cancer Network, Inc. 2024. Tutti i diritti riservati. Accesso 29 aprile 2024. Per visualizzare la versione pi\u00f9 recente e completa delle linee guida, visita il sito NCCN.org. NCCN non fornisce alcuna garanzia di alcun tipo in merito al loro contenuto, utilizzo o applicazione e declina qualsiasi responsabilit\u00e0 per la loro applicazione o il loro utilizzo in qualsiasi modo.<\/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>Panoramica della malattia Eziologia e patogenesi Principi di gestione Informazioni sui tumori desmoidi Panoramica della malattia Una malattia infiltrante e aggressiva1 I tumori desmoidi sono tumori localmente aggressivi dei tessuti molli, potenzialmente associati a morbilit\u00e0, con tendenza a infiltrare le strutture circostanti e a diffondersi lungo le fasce e i muscoli1,2 A volte indicati come&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-21936","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>Tumori desmoidi: informazioni sui tumori desmoidi | SpringWorks Therapeutics<\/title>\n<meta name=\"description\" content=\"Panoramica della malattia: esplora i principi di eziologia, patogenesi e gestione dei tumori desmoidi.\" \/>\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\/it\/informazioni-sui-tumori-desmoidi\/\" \/>\n<meta property=\"og:locale\" content=\"it_IT\" \/>\n<meta property=\"og:type\" 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