{"id":21810,"date":"2024-08-07T10:14:05","date_gmt":"2024-08-07T10:14:05","guid":{"rendered":"https:\/\/www.desmoidtumours.eu\/acerca-de-los-tumores-desmoides\/"},"modified":"2026-03-12T10:06:41","modified_gmt":"2026-03-12T10:06:41","slug":"acerca-de-los-tumores-desmoides","status":"publish","type":"page","link":"https:\/\/www.desmoidtumours.eu\/es\/acerca-de-los-tumores-desmoides\/","title":{"rendered":"Acerca de los tumores desmoides"},"content":{"rendered":"\t\t<div data-elementor-type=\"wp-page\" data-elementor-id=\"21810\" class=\"elementor elementor-21810 elementor-20245\" data-elementor-post-type=\"page\">\n\t\t\t\t<div class=\"elementor-element elementor-element-67062ba7 e-flex e-con-boxed e-con e-parent\" data-id=\"67062ba7\" 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-439f7f95 elementor-widget elementor-widget-spacer\" data-id=\"439f7f95\" 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-ab6d89e the7-e-sticky-row-yes e-flex e-con-boxed e-con e-parent\" data-id=\"ab6d89e\" 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-3b14b568 e-con-full e-flex e-con e-child\" data-id=\"3b14b568\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-4686d1f1 elementor-widget elementor-widget-text-editor\" data-id=\"4686d1f1\" 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\">Descripci\u00f3n general de la enfermedad<\/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-7baae91d e-con-full e-flex e-con e-child\" data-id=\"7baae91d\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-3434d677 elementor-widget elementor-widget-text-editor\" data-id=\"3434d677\" 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\">Etiolog\u00eda y patog\u00e9nesis<\/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-6d9b6197 e-con-full e-flex e-con e-child\" data-id=\"6d9b6197\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-3861a256 elementor-widget elementor-widget-text-editor\" data-id=\"3861a256\" 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\">Principios de manejo<\/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-bf3c070 e-con-full e-flex e-con e-parent\" data-id=\"bf3c070\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t<div class=\"elementor-element elementor-element-6060254f e-flex e-con-boxed e-con e-child\" data-id=\"6060254f\" 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-b5b0660 elementor-widget elementor-widget-heading\" data-id=\"b5b0660\" 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\">Acerca de los tumores desmoides<\/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-496ff1d7 e-con-full section e-flex e-con e-parent\" data-id=\"496ff1d7\" 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-4e1e8ae5 e-flex e-con-boxed e-con e-child\" data-id=\"4e1e8ae5\" data-element_type=\"container\">\n\t\t\t\t\t<div class=\"e-con-inner\">\n\t\t<div class=\"elementor-element elementor-element-68478bb9 e-con-full e-flex e-con e-child\" data-id=\"68478bb9\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-591de3d9 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"591de3d9\" 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\">Descripci\u00f3n general de la enfermedad:<\/h2>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-55737c01 e-con-full e-flex e-con e-child\" data-id=\"55737c01\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-7b25398f elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"7b25398f\" 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 enfermedad infiltrativa y agresiva<sup>1<\/sup><\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-7b331392 elementor-widget-tablet__width-inherit [12:16 PM] Giovanni Ferraioli bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"7b331392\" 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>Los tumores desmoides son tumores <strong>localmente agresivos<\/strong> y potencialmente m\u00f3rbidos de los tejidos blandos, con tendencia a infiltrarse en las estructuras circundantes y extenderse por los planos y los m\u00fasculos<sup>1,2<\/sup><\/li><li>A veces denominados fibromatosis agresiva o fibromatosis desmoide,<sup>3<\/sup> estos tumores mesenquimatosos pueden ser <strong>graves<\/strong>, <strong>debilitantes<\/strong> y, en casos infrecuentes, incluso causar da\u00f1os org\u00e1nicos <strong>potencialmente mortales<\/strong><sup>2,4<\/sup><\/li><li>Los tumores desmoides pueden infiltrarse en el tejido circundante, comprimiendo m\u00fasculos, nervios y vasos<sup>5,6<\/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-3d32d9db elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"3d32d9db\" 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\">Sintomatolog\u00eda y localizaciones tumorales<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-7aca6ea4 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"7aca6ea4\" 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\">Los tumores desmoides pueden causar morbilidades graves y debilitantes<sup>2<\/sup><\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-6748986f elementor-widget-tablet__width-inherit [12:16 PM] Giovanni Ferraioli bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"6748986f\" 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<li>La sintomatolog\u00eda relacionada con los tumores desmoides var\u00eda en funci\u00f3n de la localizaci\u00f3n de la presentaci\u00f3n del tumor<sup>4,6<\/sup><\/li>\n<li>Un estudio prospectivo de cohortes hall\u00f3 que los tumores de la <strong>pared tor\u00e1cica<\/strong>, las <strong>extremidades superiores<\/strong> <strong>y la cabeza y el cuello<\/strong> se asociaban con malos resultados despu\u00e9s de la cirug\u00eda<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-595689e8 elementor-widget elementor-widget-the7_button_widget\" data-id=\"595689e8\" 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=\"\/es\/experiencia-del-paciente\/#disease-burden\" class=\"box-button elementor-button elementor-size-md\">M\u00e1s informaci\u00f3n sobre el tratamiento del tumor desmoide<\/a><\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-3b72f880 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"3b72f880\" 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-9\" class=\"tablepress tablepress-id-9 first-tumors-table\">\n<thead>\n<tr class=\"row-1\">\n\t<th class=\"column-1\">LOCALIZACI\u00d3N DEL TUMOR<\/th><th class=\"column-2\">FRECUENCIA ESTIMADA<sup>6<\/sup>\u200b<\/th><th class=\"column-3\">S\u00cdNTOMAS Y COMPLICACIONES COMUNES<\/th>\n<\/tr>\n<\/thead>\n<tbody class=\"row-striping\">\n<tr class=\"row-2\">\n\t<td class=\"column-1\"><strong>Intraabdominal<\/strong><br \/><small>(including mesentery)<\/small><\/td><td class=\"column-2\"><strong>20%<\/strong><\/td><td class=\"column-3\">La compresi\u00f3n puede causar dolor, caquexia, malestar, distensi\u00f3n abdominal u obstrucci\u00f3n de los intestinos o de los ur\u00e9teres<sup>6,8<\/sup><\/td>\n<\/tr>\n<tr class=\"row-3\">\n\t<td class=\"column-1\"><strong>Pared Abdominal<\/strong><\/td><td class=\"column-2\"><strong>16%<\/strong><\/td><td class=\"column-3\">Los tumores de gran tama\u00f1o pueden provocar estiramiento de los tejidos, compresi\u00f3n de los vasos sangu\u00edneos y desplazamiento del intestino o de la vejiga<sup>9<\/sup><\/td>\n<\/tr>\n<tr class=\"row-4\">\n\t<td class=\"column-1\"><strong>Extremidades Inferiores<\/strong><\/td><td class=\"column-2\"><strong>16%<\/strong><\/td><td class=\"column-3\">Movilidad limitada, dolor, rigidez muscular o deformidad<sup>6,10<\/sup><\/td>\n<\/tr>\n<tr class=\"row-5\">\n\t<td class=\"column-1\"><strong>Pared Tor\u00e1cica<\/strong><\/td><td class=\"column-2\"><strong>15%<\/strong><\/td><td class=\"column-3\">Disnea,<sup>6<\/sup> disfagia,<sup>6<\/sup> invasi\u00f3n pleural,<sup>8<\/sup> fectaci\u00f3n de la columna vertebral<sup>11<\/sup> o de las costillas,<sup>12<\/sup> erosi\u00f3n \u00f3sea,<sup>12<\/sup> y dolor<sup>12<\/sup><\/td>\n<\/tr>\n<tr class=\"row-6\">\n\t<td class=\"column-1\"><strong>Extremidades Superiores<\/strong><\/td><td class=\"column-2\"><strong>14%<\/strong><\/td><td class=\"column-3\">Movilidad restringida, afectaci\u00f3n de m\u00fasculos y ligamentos, debilidad del miembro, deformidad o dolor<sup>6,13<\/sup><\/td>\n<\/tr>\n<tr class=\"row-7\">\n\t<td class=\"column-1\"><strong>Cabeza Y Cuello<\/strong><\/td><td class=\"column-2\"><strong>8%<\/strong><\/td><td class=\"column-3\">Dolor, d\u00e9ficit neurol\u00f3gico, proximidad a estructuras vitales, incluido el riesgo de mortalidad por compromiso vascular o de las v\u00edas respiratorias<sup>14<\/sup><\/td>\n<\/tr>\n<tr class=\"row-8\">\n\t<td class=\"column-1\"><strong>Otros<\/strong><\/td><td class=\"column-2\"><strong>11%<\/strong><\/td><td class=\"column-3\">S\u00edntomas y complicaciones dependientes de la localizaci\u00f3n<sup>4,6<\/sup><\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<!-- #tablepress-9 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-2b32b206 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"2b32b206\" 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 ubicaci\u00f3n de los tumores desmoides puede afectar significativamente a la calidad de vida.<\/strong><sup>15<\/sup> Por ejemplo, un tumor desmoide con crecimientos \u201csimilares a tendriles\u201d<sup>1<\/sup> que rodean los <strong>nervios<\/strong> puede estar asociado a <strong>dolor neurop\u00e1tico debilitante<\/strong>.<sup>6,16<\/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-7445d26a elementor-widget elementor-widget-the7_button_widget\" data-id=\"7445d26a\" 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=\"\/es\/experiencia-del-paciente\/#disease-burden\" class=\"box-button elementor-button elementor-size-md\">M\u00e1s informaci\u00f3n sobre la carga de los tumores desmoides<\/a><\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-357dd5c4 e-grid e-con-full e-con e-child\" data-id=\"357dd5c4\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-68fb815f e-con-full e-flex e-con e-child\" data-id=\"68fb815f\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-7114a5cd elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"7114a5cd\" 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\">Evoluci\u00f3n de la enfermedad<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-4e954e4d elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"4e954e4d\" 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 e impredecible<sup>1<\/sup><\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-24a2e44b elementor-widget-tablet__width-inherit [12:16 PM] Giovanni Ferraioli bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"24a2e44b\" 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>Los tumores desmoides se caracterizan por una evoluci\u00f3n variable e impredecible de la enfermedad dependiendo de la localizaci\u00f3n del tumor y de la morbilidad asociada<sup>1,6<\/sup><\/li><li>Aproximadamente, el 50 % de los desmoides presentan una biolog\u00eda agresiva, continuando su crecimiento o volvi\u00e9ndose sintom\u00e1ticos. La gran mayor\u00eda de las progresiones (89 %) se produjeron en los primeros 2 a\u00f1os de observaci\u00f3n<sup>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<div class=\"elementor-element elementor-element-2a6cd365 e-con-full e-flex e-con e-child\" data-id=\"2a6cd365\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-3dcf9336 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=\"3dcf9336\" 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-3dcf9336\">\t\t\t<div class=\"box-content-wrapper\">\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 >Evoluci\u00f3n cl\u00ednica de los tumores desmoides<\/a><\/h6><div class=\"box-description\">Elaborado a partir del texto, referencia 17.<br \/>\n*El 10\u201328 % de los tumores desmoides se resolver\u00e1n\nespont\u00e1neamente sin tratamiento<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-59893ba elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"59893ba\" 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\">Incidencia y factores de riesgo<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-3b5238ba e-con-full e-flex e-con e-child\" data-id=\"3b5238ba\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-131aaef9 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=\"131aaef9\" 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-131aaef9\">\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<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=\"Epidemiolog\u00eda\">\t\t\t\t\t\t\t\t\t\t\t\tEpidemiolog\u00eda\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>El tumor desmoide tiene una incidencia de aproximadamente <strong>5 casos por cada mill\u00f3n de habitantes<\/strong> al a\u00f1o.<sup>18<\/sup><\/p>\n<p>La serie de informes Orphanet proporciona una incidencia anual media de 3 casos de tumores desmoides por mill\u00f3n de a\u00f1os-persona en Europa.<sup>17<\/sup><\/p>\n<p>Los estudios epidemiol\u00f3gicos revelan que la mayor\u00eda de los casos de tumores desmoides aparecen en el <strong>intervalo de edad de 20 a 44 a\u00f1os<\/strong>.<sup>17<\/sup><\/p>\n<ul>\n \t<li>Los tumores desmoides son infrecuentes en personas j\u00f3venes y adultos de edad avanzada<sup>.19<\/sup><\/li>\n<li>La estimaci\u00f3n de la incidencia ha aumentado en los \u00faltimos a\u00f1os, lo que puede estar relacionado con una mejora en las t\u00e9cnicas diagn\u00f3sticas<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<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=\"Sexo femenino\">\t\t\t\t\t\t\t\t\t\t\t\tSexo femenino\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>La proporci\u00f3n entre mujeres y hombres es de aproximadamente 2-3:1.<sup>20<\/sup><\/p>\n<ul>\n<li>El embarazo puede promover tumores desmoides<sup>5<\/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<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=\"Traumatismo antecedente\">\t\t\t\t\t\t\t\t\t\t\t\tTraumatismo antecedente\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>Alrededor del 25 % de los pacientes con tumores desmoides tienen antecedentes de traumatismos.<sup>6<\/sup>\n<\/p>\n<ul>\n<li>Las lesiones o la cirug\u00eda pueden aumentar el riesgo<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<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=\"Mutaciones del APC (gen de la poliposis adenomatosa coli)\">\t\t\t\t\t\t\t\t\t\t\t\tMutaciones del APC (gen de la poliposis adenomatosa 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><strong>Las caracter\u00edsticas fenot\u00edpicas<\/strong> se correlacionan con la posici\u00f3n de la mutaci\u00f3n del APC en relaci\u00f3n con determinados codones.<sup>22<\/sup>\n<\/p>\n<ul>\n \t<li>Los pacientes con poliposis adenomatosa familiar (PAF) tienen un riesgo 1000 veces mayor de desarrollar tumores desmoides que la poblaci\u00f3n general<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-20b56432 e-con-full section e-flex e-con e-parent\" data-id=\"20b56432\" 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-29fd4834 e-flex e-con-boxed e-con e-child\" data-id=\"29fd4834\" data-element_type=\"container\">\n\t\t\t\t\t<div class=\"e-con-inner\">\n\t\t<div class=\"elementor-element elementor-element-41a513ff e-con-full e-flex e-con e-child\" data-id=\"41a513ff\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-2a971250 e-con-full e-flex e-con e-child\" data-id=\"2a971250\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-7055af1d elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"7055af1d\" 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\">Etiolog\u00eda y patog\u00e9nesis de los tumores desmoides <\/h2>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-7520a9d0 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"7520a9d0\" 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\">Comprender la patogenia molecular es de gran inter\u00e9s en esta enfermedad, dada su gran variabilidad en su evoluci\u00f3n cl\u00ednica<sup>19<\/sup><\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-1acd35d6 elementor-widget-tablet__width-inherit [12:16 PM] Giovanni Ferraioli bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"1acd35d6\" 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\tSe desconoce la etiolog\u00eda de los tumores desmoides. Sin embargo, la identificaci\u00f3n de cambios cromos\u00f3micos clonales en una fracci\u00f3n significativa de los casos respalda la naturaleza neopl\u00e1sica de estos tumores.<sup>19<\/sup>\n<ul>\n \t<li>Se cree que la patog\u00e9nesis de los tumores desmoides est\u00e1 relacionada con un traumatismo<sup>5<\/sup>\n<ul>\n \t<li>a veces la cirug\u00eda puede promover el crecimiento del tumor<sup style=\"word-spacing: normal;\">5<\/sup><\/li>\n \t<li>factores de crecimiento liberados despu\u00e9s de la cirug\u00eda, durante la fase inicial de la cicatrizaci\u00f3n de heridas, podr\u00edan transmitir se\u00f1ales que promueven la activaci\u00f3n de la \u03b2-catenina<sup>5<\/sup><\/li>\n<\/ul>\n<\/li>\n \t<li>El embarazo puede promover el TD no solo secundario a las influencias hormonales, sino tambi\u00e9n a la liberaci\u00f3n sist\u00e9mica de factores de crecimiento<sup>5<\/sup><\/li>\n \t<li>Se han implicado dos v\u00edas en la v\u00eda can\u00f3nica Wnt\/\u03b2-catenina\/APC, en la que las mutaciones en los genes CTNNB1 y APC provocan la acumulaci\u00f3n de \u03b2-catenina, y la v\u00eda Notch, en la que se producen dos escisiones en los receptores de Notch <sup>17<\/sup>\n<ul>\n \t<li>Se cree que la posible interferencia entre las v\u00edas de Notch y Wnt, as\u00ed como la activaci\u00f3n de la v\u00eda Notch resultante de la desregulaci\u00f3n de la v\u00eda Wnt, est\u00e1n implicadas en la patog\u00e9nesis de los tumores desmoides<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-70fe5e38 e-grid e-con-full e-con e-child\" data-id=\"70fe5e38\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-4935a410 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=\"4935a410\" 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-4935a410\">\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=\"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\/2026\/03\/Nuclear-accumulation-of-beta-catenin.png\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/03\/Nuclear-accumulation-of-beta-catenin.png 508w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/03\/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 >Acumulaci\u00f3n nuclear de \u03b2-catenina<\/a><\/h6><div class=\"box-description\"><ul>\n \t<li>La desregulaci\u00f3n de la v\u00eda Wnt\/\u03b2-catenina se ha relacionado con la patog\u00e9nesis de los tumores desmoides<sup>2,23<\/sup><\/li>\n \t<li>La acumulaci\u00f3n nuclear de \u03b2-catenina y la subsiguiente\ntranscripci\u00f3n g\u00e9nica son un factor clave que impulsa la proliferaci\u00f3n de c\u00e9lulas tumorales desmoides<sup>2,24<\/sup><\/li>\n \t<li>La acumulaci\u00f3n nuclear de \u03b2-catenina puede iniciarse por<sup>2,24<\/sup>\n<ul>\n \t<li>Mutaciones activadoras del gen de la \u03b2-catenina <em>CTNNB1<\/em><\/li>\n \t<li>Mutaciones inactivadoras en el <em>APC<\/em> regulador negativo, a menudo en poliposis adenomatosa familiar (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-7aacf476 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=\"7aacf476\" 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-7aacf476\">\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\/2026\/03\/Notch-activation-by-gamma-secretase-copy-1.jpg\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/03\/Notch-activation-by-gamma-secretase-copy-1.jpg 512w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/03\/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 >Activaci\u00f3n de Notch por la \u03b3-secretasa<\/a><\/h6><div class=\"box-description\"><ul>\n \t<li>La v\u00eda Notch puede estar activa en los tumores desmoides<sup>25<\/sup><\/li>\n \t<li>Cuando se desregula, Notch puede activar v\u00edas que contribuyen al crecimiento tumoral<sup>25<\/sup><\/li>\n \t<li>La se\u00f1alizaci\u00f3n del receptor de Notch requiere la activaci\u00f3n proteol\u00edtica por la enzima \u03b3-secretasa (gamma secretasa)<sup>25<\/sup><\/li>\n \t<li>La escisi\u00f3n de la \u03b3-secretasa libera el dominio intracelular de Notch (DICN), que se transloca al n\u00facleo y activa la transcripci\u00f3n g\u00e9nica<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-7a7f42e 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=\"7a7f42e\" 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-7a7f42e\">\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\/2026\/03\/Patchway-crosstalk.jpg\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/03\/Patchway-crosstalk.jpg 508w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/03\/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 >Intercomunicaci\u00f3n de la v\u00eda<\/a><\/h6><div class=\"box-description\"><ul>\n \t<li>La interferencia entre las v\u00edas Wnt\/\u03b2-catenina y Notch puede contribuir a\u00fan m\u00e1s a la patogenia tumoral 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-1621521c 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=\"1621521c\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-31ec6dd6 e-con-full e-flex e-con e-child\" data-id=\"31ec6dd6\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-3d77889e 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=\"3d77889e\" 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-3d77889e\">\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=\"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\/2026\/03\/Nuclear-accumulation-of-beta-catenin.png\" loading=\"eager\" data-srcset=\"https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/03\/Nuclear-accumulation-of-beta-catenin.png 508w, https:\/\/www.desmoidtumours.eu\/wp-content\/uploads\/2026\/03\/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 >Acumulaci\u00f3n nuclear de \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-56237bb3 e-con-full e-flex e-con e-child\" data-id=\"56237bb3\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-58500b3f elementor-widget-tablet__width-inherit bullet-custom  elementor-widget elementor-widget-text-editor\" data-id=\"58500b3f\" 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 desregulaci\u00f3n de la v\u00eda Wnt\/\u03b2-catenina se ha relacionado con la patog\u00e9nesis de los tumores desmoides<sup>2,24<\/sup><\/li>\n \t<li>La acumulaci\u00f3n nuclear de \u03b2-catenina y la subsiguiente\ntranscripci\u00f3n g\u00e9nica son un factor clave que impulsa la proliferaci\u00f3n de c\u00e9lulas tumorales desmoides<sup>2,25<\/sup><\/li>\n \t<li>La acumulaci\u00f3n nuclear de \u03b2-catenina puede iniciarse por<sup>2,25<\/sup>\n<ul>\n \t<li>Mutaciones activadoras del gen de la \u03b2-catenina <em>CTNNB1<\/em><\/li>\n \t<li>Mutaciones inactivadoras en el <em>APC<\/em> regulador negativo, a menudo en poliposis adenomatosa familiar (PAF)<\/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-19b3becd 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=\"19b3becd\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-5d21ba20 e-con-full e-flex e-con e-child\" data-id=\"5d21ba20\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-4e184b1d 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=\"4e184b1d\" 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-4e184b1d\">\t\t\t<div class=\"box-content-wrapper\">\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 >Activaci\u00f3n de Notch por la \u03b3-secretasa<\/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-b4a0829 e-con-full e-flex e-con e-child\" data-id=\"b4a0829\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-6ca7b653 elementor-widget-tablet__width-inherit bullet-custom  elementor-widget elementor-widget-text-editor\" data-id=\"6ca7b653\" 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 v\u00eda Notch puede estar activa en los tumores desmoides<sup>26<\/sup><\/li>\n \t<li>Cuando se desregula, Notch puede activar v\u00edas que contribuyen al crecimiento tumoral<sup>26<\/sup><\/li>\n \t<li>La se\u00f1alizaci\u00f3n del receptor Notch requiere la activaci\u00f3n proteol\u00edtica por la enzima <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>-secretasa<sup>26<\/sup><\/li>\n \t<li>La escisi\u00f3n de la <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>-secretasa libera el dominio intracelular de Notch (DICN), que se transloca al n\u00facleo y activa la transcripci\u00f3n g\u00e9nica<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-3cc27ab 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=\"3cc27ab\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-4368be05 e-con-full e-flex e-con e-child\" data-id=\"4368be05\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-1b5031dd 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=\"1b5031dd\" 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-1b5031dd\">\t\t\t<div class=\"box-content-wrapper\">\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 >Intercomunicaci\u00f3n de la v\u00eda<\/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-4d414452 e-con-full e-flex e-con e-child\" data-id=\"4d414452\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-464042b5 elementor-widget-tablet__width-inherit bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"464042b5\" 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 interferencia entre las v\u00edas Wnt\/\u03b2-catenina y Notch puede contribuir a\u00fan m\u00e1s a la patogenia tumoral 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-35025333 elementor-widget elementor-widget-menu-anchor\" data-id=\"35025333\" 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-2cb916cd e-con-full e-flex e-con e-child\" data-id=\"2cb916cd\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-4a512ec5 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"4a512ec5\" 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\">Riesgo de recidiva<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-2aee9b84 elementor-widget-tablet__width-inherit bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"2aee9b84\" 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>Las tasas de recidiva pueden verse influidas por la localizaci\u00f3n del tumor:<sup>17<\/sup><ul><li>Se cree que los tumores de las extremidades son localmente agresivos y tienen tasas de recidiva que van del 24 % al 77 %<\/li><li>Las tasas de recidiva local de tumores intraabdominales en pacientes con PAF son superiores a las de los tumores extraabdominales y se notific\u00f3 que eran del 57 % al 86 %<\/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-400ea637 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"400ea637\" 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\">Existe un alto riesgo de recidiva local despu\u00e9s de la cirug\u00eda<sup>17<\/sup><\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-4da9aea4 elementor-widget-tablet__width-inherit bullet-custom   elementor-widget elementor-widget-text-editor\" data-id=\"4da9aea4\" 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>Las tasas de recidiva pueden exacerbarse por traumatismos, como traumatismos quir\u00fargicos, y oscilan aproximadamente entre el 25 % y el 60 % a los 5 a\u00f1os<sup>17<\/sup>\n<ul>\n \t<li>La invasi\u00f3n de los principales vasos y nervios y la calidad de los bordes quir\u00fargicos son factores clave para la elevada tasa de recidiva posoperatoria<sup>26<\/sup><\/li>\n \t<li>Desafortunadamente, incluso cuando los bordes quir\u00fargicos no presentan tumores, las tasas de recidiva son altas<sup>27<\/sup><\/li>\n<\/ul>\n<\/li>\n<\/ul>\nLos factores de crecimiento liberados despu\u00e9s de la cirug\u00eda durante la fase inicial de cicatrizaci\u00f3n de heridas podr\u00edan promover la activaci\u00f3n de la \u03b2-catenina.<sup>5<\/sup>\n<ul>\n \t<li>El hecho de que algunos tumores reaparecieron despu\u00e9s de la cirug\u00eda pero luego permanecieron estables sin tratamiento indica que los factores de crecimiento liberados despu\u00e9s de la cirug\u00eda pueden haber fomentado la recidiva en tumores que de otro modo habr\u00edan sido de escasa malignidad<sup>5<\/sup><\/li>\n \t<li>La mutaci\u00f3n espec\u00edfica S45F en el gen CTNNB1 parece estar asociada con una peor supervivencia sin recidiva despu\u00e9s de la cirug\u00eda, tal como han sugerido m\u00faltiples cohortes retrospectivas<sup>28<\/sup>\n<ul>\n \t<li>La supervivencia sin recidiva a los 5 a\u00f1os fue significativamente menor en pacientes con tumores desmoides que portaban la mutaci\u00f3n S45F (p &lt; 0,0001) en comparaci\u00f3n con pacientes con tumores con la mutaci\u00f3n T41A o sin mutaci\u00f3n<sup>29<\/sup>. Dado que estos resultados proceden de estudios observacionales, es importante interpretarlos considerando la variabilidad entre cohortes y factores como el sitio tumoral y el manejo aplicado.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-6d2f739 e-con-full e-flex e-con e-child\" data-id=\"6d2f739\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-752501b3 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"752501b3\" 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-10\" class=\"tablepress tablepress-id-10 tumors-table\">\n<thead>\n<tr class=\"row-1\">\n\t<th class=\"column-1\">MUTACI\u00d3N<\/th><th class=\"column-2\">SUPERVIVENCIA LIBRE DE RECURRENCIA ESTIMADA (IC 95%)<\/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 mutado<\/td><td class=\"column-2\">65% (38%-83%)<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<!-- #tablepress-10 from cache -->\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-4c65d75 e-con-full e-flex e-con e-child\" data-id=\"4c65d75\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t<div class=\"elementor-element elementor-element-489bf15c e-con-full e-flex e-con e-child\" data-id=\"489bf15c\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-49961d2e e-con-full e-flex e-con e-child\" data-id=\"49961d2e\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-59c599a6 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=\"59c599a6\" 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-59c599a6\">\t\t\t<div class=\"box-content-wrapper\">\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 >Los pacientes con mutaciones en CTNNB1 en el cod\u00f3n 45 (45F) presentaron un riesgo 3 veces mayor de recidiva despu\u00e9s de la cirug\u00eda<sup>29<\/sup><\/a><\/h6><div class=\"box-description\">Elaborado a partir de la figura 2b, referencia 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-56a3f59c elementor-widget elementor-widget-text-editor\" data-id=\"56a3f59c\" 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>En un metan\u00e1lisis, se notific\u00f3 que el tama\u00f1o del tumor era un mediador importante del aumento del riesgo de recidiva en pacientes con la mutaci\u00f3n S45F en el gen CTNNB1.<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-457bd0b2 elementor-widget elementor-widget-the7_button_widget\" data-id=\"457bd0b2\" 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=\"\/es\/abordaje\/#surgery\" class=\"box-button elementor-button elementor-size-md\">Informaci\u00f3n sobre consideraciones quir\u00fargicas<\/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-45d5ba6c e-con-full section e-flex e-con e-parent\" data-id=\"45d5ba6c\" 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-337ef9c e-flex e-con-boxed e-con e-child\" data-id=\"337ef9c\" data-element_type=\"container\">\n\t\t\t\t\t<div class=\"e-con-inner\">\n\t\t<div class=\"elementor-element elementor-element-36cbc7b1 e-con-full e-flex e-con e-child\" data-id=\"36cbc7b1\" data-element_type=\"container\">\n\t\t<div class=\"elementor-element elementor-element-2d5b37b1 e-con-full e-flex e-con e-child\" data-id=\"2d5b37b1\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-4773044e elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"4773044e\" 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\">Principios de la gesti\u00f3n<\/h2>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-6ce1c258 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"6ce1c258\" 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\">Es imprescindible seleccionar cuidadosamente la estrategia de tratamiento para cada paciente con tumores desmoides para optimizar el control tumoral y mejorar la calidad de vida<sup>18<\/sup><\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-371ba03c elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"371ba03c\" 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>En la \u00faltima d\u00e9cada se han realizado esfuerzos importantes para estandarizar el tratamiento de esta enfermedad.<sup>18<\/sup><\/p><p>Actualmente, las necesidades no cubiertas en los tumores desmoides incluyen un diagn\u00f3stico temprano y preciso y tratamientos aprobados indicados para pacientes con tumores desmoides.<sup>17<\/sup><\/p><p>En un estudio realizado en el Memorial Sloan Kettering Cancer Center, el 58 % de los pacientes con tumores desmoides pasaron de vigilancia activa a tratamiento de primera l\u00ednea sin progresi\u00f3n radiogr\u00e1fica del tumor.<sup>31\u2020<\/sup><\/p><p>Los objetivos del tratamiento no deben centrarse \u00fanicamente en los marcadores cl\u00ednicos, como la supervivencia sin progresi\u00f3n, sino tambi\u00e9n considerar los criterios de valoraci\u00f3n relevantes para el paciente, como la reducci\u00f3n de la carga de s\u00edntomas espec\u00edficos del tumor desmoide (por ejemplo, el dolor) y su impacto en la vida de los pacientes, la mejora del funcionamiento en las actividades diarias y la calidad de vida en general.<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-7582cf elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"7582cf\" 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\">Identificaci\u00f3n temprana de la progresi\u00f3n<\/h3>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-306e04a9 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"306e04a9\" 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 identificaci\u00f3n de la progresi\u00f3n del tumor desmoide puede dar lugar al inicio oportuno del tratamiento adecuado<sup>32,33<\/sup><\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-77936d86 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"77936d86\" 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>Para ayudar a mejorar los resultados de los pacientes, los profesionales sanitarios deben <strong>evaluar la progresi\u00f3n lo antes posible<\/strong> con al menos uno de los siguientes:<\/li><\/ul>\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-74383f58 e-con-fit e-flex e-con e-child\" data-id=\"74383f58\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t\t\t<div class=\"elementor-element elementor-element-3393af87 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=\"3393af87\" 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-3393af87\">\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<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=\"Crecimiento tumoral documentado mediante RM o TAC&lt;sup&gt;32,33&lt;\/sup&gt;\">\t\t\t\t\t\t\t\t\t\t\t\tCrecimiento tumoral documentado mediante RM o TAC<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>O<\/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<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=\"Empeoramiento de los s\u00edntomas&lt;sup&gt;32,33&lt;\/sup&gt;\">\t\t\t\t\t\t\t\t\t\t\t\tEmpeoramiento de los s\u00edntomas<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>O<\/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<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=\"Empeoramiento del impacto en la vida diaria (p. ej., deterioro funcional)&lt;sup&gt;32,33&lt;\/sup&gt;\">\t\t\t\t\t\t\t\t\t\t\t\tEmpeoramiento del impacto en la vida diaria (p. ej., deterioro funcional)<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-2410db83 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"2410db83\" 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>El dolor puede ser un indicador pron\u00f3stico de progresi\u00f3n<\/strong><sup>34<\/sup> y puede asociarse <strong>a peores resultados<\/strong><sup>35<\/sup><\/li><li><strong>El riesgo de progresi\u00f3n puede ser mayor para tumores m\u00e1s grandes<\/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-6d10b51c elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"6d10b51c\" 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>Los pacientes con tumores desmoides primarios o recidivantes (n = 160) se identificaron de forma retrospectiva a partir de una base de datos institucional. Entre los pacientes en observaci\u00f3n inicial para los que se dispon\u00eda de RM seriadas, 14 de 24 (58 %) de los que se sometieron a tratamiento activo no experimentaron crecimiento tumoral seg\u00fan los criterios RECIST.<sup>31<\/sup> Elaborado a partir del texto, referencia 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-27fdd0c5 elementor-widget elementor-widget-the7_button_widget\" data-id=\"27fdd0c5\" 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=\"\/es\/abordaje\/#disease-progression-identification\" class=\"box-button elementor-button elementor-size-md\">Conozca las opciones de tratamiento tras la identificaci\u00f3n temprana de la progresi\u00f3n tumoral desmoide<\/a><\/div>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-4ceb5afc elementor-widget-tablet__width-inherit elementor-widget elementor-widget-the7-heading\" data-id=\"4ceb5afc\" 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\">Escuche las perspectivas de un onc\u00f3logo m\u00e9dico sobre los tumores desmoides<\/h4>\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t<div class=\"elementor-element elementor-element-214db202 e-grid e-con-full e-con e-child\" data-id=\"214db202\" data-element_type=\"container\" id=\"video-grid\">\n\t\t\t\t<div class=\"elementor-element elementor-element-43bcf85a elementor-widget elementor-widget-video\" data-id=\"43bcf85a\" 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-1d6caab2 elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"1d6caab2\" 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>Comprender los tumores desmoides<\/h5><p>Vea c\u00f3mo el Dr. Riedel, onc\u00f3logo m\u00e9dico del Duke Health y Duke Cancer Institute, proporciona una visi\u00f3n general de los tumores desmoides, incluida su fisiopatolog\u00eda, presentaciones cl\u00ednicas e impacto en los pacientes.<\/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-1663bbe elementor-widget-tablet__width-inherit elementor-widget elementor-widget-text-editor\" data-id=\"1663bbe\" 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, gen Adenomatosis Polyposis Coli; TAC, tomograf\u00eda computarizada; CTNNB1, gen de la \u03b2-catenina 1; PAF, poliposis adenomatosa familiar; RM, resonancia magn\u00e9tica; DICN, dominio intracelular de Notch; RECIST, Criterios de evaluaci\u00f3n de la respuesta en tumores s\u00f3lidos; S45F, tipo de mutaci\u00f3n; T41A, tipo de mutaci\u00f3n; Wnt, sitio de integraci\u00f3n relacionado con 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-16b1e6eb e-con-full e-flex e-con e-parent\" data-id=\"16b1e6eb\" data-element_type=\"container\" data-settings=\"{&quot;background_background&quot;:&quot;classic&quot;}\">\n\t\t<div class=\"elementor-element elementor-element-36491913 e-flex e-con-boxed e-con e-child\" data-id=\"36491913\" data-element_type=\"container\">\n\t\t\t\t\t<div class=\"e-con-inner\">\n\t\t<div class=\"elementor-element elementor-element-fe769f2 e-con-full e-flex e-con e-child\" data-id=\"fe769f2\" data-element_type=\"container\">\n\t\t\t\t<div class=\"elementor-element elementor-element-1b45fe43 elementor-widget elementor-widget-the7-accordion\" data-id=\"1b45fe43\" 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-4571\" class=\"elementor-tab-title the7-accordion-header deactive-default\" data-tab=\"1\" role=\"tab\" aria-controls=\"elementor-tab-content-4571\">\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=\"\">Referencias<\/a>\n\t\t\t\t\t<\/h5>\n\t\t\t\t\t<div id=\"elementor-tab-content-4571\" class=\"elementor-tab-content elementor-clearfix deactive-default\" data-tab=\"1\" role=\"tabpanel\" aria-labelledby=\"elementor-tab-title-4571\"><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. doi: 10.1093\/annonc\/mdx323.<\/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. doi: 10.1097\/CCO.0000000000000374.<\/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. doi: 10.1016\/j.ejca.2019.11.013.<\/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. doi: 10.3390\/cancers3033143.<\/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. doi: 10.1093\/annonc\/mds298.<\/li>\n<li>Constantinidou A et al. Clinical presentation of desmoid tumors. En: Litchman C, ed. Desmoid Tumors. Springer; 2012:cap. 2. Consultado el 9 de abril de 2024. <a href=\"https:\/\/www.researchgate.net\/publication\/226455135\" target=\"_blank\" rel=\"noopener\">https:\/\/www.researchgate.net\/publication\/226455135<\/a>. doi: 10.1007\/978-94-007-1685-8_2<\/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. doi: 10.1016\/j.ejca.2017.06.017.<\/li>\n<li>Shinagare AB et al. A to Z of desmoid tumors. <em>AJR Am J Roentgenol<\/em>. 2011;197(6):W1008-W1014. doi: 10.2214\/AJR.11.6657.<\/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. doi: 10.1155\/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. doi: 10.1148\/rg.283075169.<\/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. doi: 10.1590\/s1807-59322011000400028.<\/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. doi: 10.3892\/ol.2014.2431.<\/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. doi: 10.1007\/s12105-019-01104-3.<\/li>\n<li>Ingley KM et al. High prevalence of persistent emotional distress in desmoid tumor.&nbsp;<em>Psychooncology<\/em>. 2020;29(2):311-320. doi: 10.1002\/pon.5250.<\/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. doi: 10.1002\/cncr.32555.<\/li>\n<li>Bektas M et al. Desmoid tumors: a comprehensive review. <em>Adv Ther<\/em>. 2023;40(9):3697-3722. doi: 10.1007\/s12325-023-02592-0.<\/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. doi: 10.1001\/jamaoncol.2024.1805.<\/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. doi: 10.1016\/j.mayocp.2017.02.012.<\/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. doi: 10.1016\/s0002-9610(05)81243-7.<\/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. doi: 10.1001\/jamaoncol.2024.1805.<\/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. doi: 10.1002\/gcc.22272.<\/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. doi: 10.1016\/j.ejca.2019.11.013.<\/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. doi: 10.1002\/cncr.29564.<\/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. doi: 10.1186\/s12957-015-0450-8.<\/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. doi: 10.1097\/00000658-198912000-00012.<\/li>\n<li>Napolitano A et al. Recent advances in desmoid tumor therapy. <em>Cancers<\/em> (<em>Basel<\/em>). 2020;12(8):2135.&nbsp;doi: 10.3390\/cancers12082135.<\/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. doi: 10.2353\/ajpath.2008.080475.<\/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. doi: 10.1097\/SLA.0000000000003698.<\/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. doi: 10.1097\/SLA.0000000000003073.<\/li>\n<li>Tomado como referencia con permiso de NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines\u00ae) for Soft Tissue Sarcoma V.1.2024. \u00a9 National Comprehensive Cancer Network, Inc. 2024. Todos los derechos reservados. Consultado el 29 de abril de 2024. Para ver la versi\u00f3n m\u00e1s reciente y completa de las directrices, visite NCCN.org. La NCCN no ofrece ninguna garant\u00eda de ning\u00fan tipo en relaci\u00f3n con su contenido, uso o aplicaci\u00f3n y rechaza cualquier responsabilidad sobre su aplicaci\u00f3n o uso de cualquier forma.<\/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. doi: 10.1001\/jamaoncol.2024.1805.<\/li>\n<li>Cuomo P et al. Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study. <em>BMC Cancer<\/em>. 2021;21(1):437. doi: 10.1186\/s12885-021-08189-6.<\/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. doi: 10.1002\/ijc.34493.<\/li>\n<\/ol><\/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>Descripci\u00f3n general de la enfermedad Etiolog\u00eda y patog\u00e9nesis Principios de manejo Acerca de los tumores desmoides Descripci\u00f3n general de la enfermedad: Una enfermedad infiltrativa y agresiva1 Los tumores desmoides son tumores localmente agresivos y potencialmente m\u00f3rbidos de los tejidos blandos, con tendencia a infiltrarse en las estructuras circundantes y extenderse por los planos y los&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":true,"footnotes":""},"class_list":["post-21810","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>Tumores desmoides: Acerca de los tumores desmoides | SpringWorks Therapeutics<\/title>\n<meta name=\"description\" content=\"Descripci\u00f3n general de la enfermedad: Explore la etiolog\u00eda, la patog\u00e9nesis y los principios de tratamiento de los tumores desmoides.\" \/>\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\/es\/acerca-de-los-tumores-desmoides\/\" \/>\n<meta property=\"og:locale\" content=\"es_ES\" 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