{"id":7858,"date":"2020-04-26T04:11:53","date_gmt":"2020-04-26T07:11:53","guid":{"rendered":"https:\/\/doctorhoogstra.com\/es\/dermatofibrosarcoma-protuberans\/"},"modified":"2020-04-27T15:15:11","modified_gmt":"2020-04-27T18:15:11","slug":"dermatofibrosarcoma-protuberans-2","status":"publish","type":"wiki","link":"https:\/\/doctorhoogstra.com\/en\/wiki\/dermatofibrosarcoma-protuberans\/","title":{"rendered":"Dermatofibrosarcoma protuberans"},"content":{"rendered":"<p><\/p><div id=\"ez-toc-container\" class=\"ez-toc-v2_0_88 counter-hierarchy ez-toc-counter ez-toc-grey ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Contents<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Toggle Table of Content\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Toggle<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewbox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" fill=\"currentColor\"><\/path><\/svg><svg style=\"fill: #999;color:#999\" class=\"arrow-unsorted-368013\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"10px\" height=\"10px\" viewbox=\"0 0 24 24\" version=\"1.2\" baseprofile=\"tiny\"><path d=\"M18.2 9.3l-6.2-6.3-6.2 6.3c-.2.2-.3.4-.3.7s.1.5.3.7c.2.2.4.3.7.3h11c.3 0 .5-.1.7-.3.2-.2.3-.5.3-.7s-.1-.5-.3-.7zM5.8 14.7l6.2 6.3 6.2-6.3c.2-.2.3-.5.3-.7s-.1-.5-.3-.7c-.2-.2-.4-.3-.7-.3h-11c-.3 0-.5.1-.7.3-.2.2-.3.5-.3.7s.1.5.3.7z\"\/><\/svg><\/span><\/span><\/span><\/a><\/span><\/div>\n<nav><ul class='ez-toc-list ez-toc-list-level-1 eztoc-toggle-hide-by-default' ><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/dermatofibrosarcoma-protuberans\/#%C2%BFQue-es-el-dermatofibrosarcoma-protuberante\" >What is dermatofibrosarcoma protuberant?<\/a><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/dermatofibrosarcoma-protuberans\/#Dermatofibrosarcoma-protuberans\" >Dermatofibrosarcoma protuberans<\/a><\/li><\/ul><\/li><\/ul><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/dermatofibrosarcoma-protuberans\/#%C2%BFQue-causa-el-dermatofibrosarcoma-protuberans\" >What causes dermatofibrosarcoma protuberans?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/dermatofibrosarcoma-protuberans\/#%C2%BFQuien-esta-en-riesgo-de-dermatofibrosarcoma-protuberans\" >Who is at risk for dermatofibrosarcoma protuberans?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/dermatofibrosarcoma-protuberans\/#%C2%BFCuales-son-los-signos-y-sintomas-del-dermatofibrosarcoma-protuberante\" >What are the signs and symptoms of dermatofibrosarcoma protruding?<\/a><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/dermatofibrosarcoma-protuberans\/#Mas-ejemplos-de-dermatofibrosarcoma-protuberans\" >More examples of dermatofibrosarcoma protuberans<\/a><\/li><\/ul><\/li><\/ul><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/dermatofibrosarcoma-protuberans\/#%C2%BFComo-se-diagnostica-el-dermatofibrosarcoma-protuberans\" >How is dermatofibrosarcoma protuberans diagnosed?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/dermatofibrosarcoma-protuberans\/#%C2%BFCual-es-el-tratamiento-para-dermatofibrosarcoma-protuberans\" >What is the treatment for dermatofibrosarcoma protuberans?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/dermatofibrosarcoma-protuberans\/#Cual-es-el-pronostico-para-dermatofibrosarcoma-protuberans\" >What is the prognosis for dermatofibrosarcoma protuberans?<\/a><\/li><\/ul><\/nav><\/div>\n\n<section class=\"textBlock\">\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQue-es-el-dermatofibrosarcoma-protuberante\"><\/span>What is dermatofibrosarcoma protuberant?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Dermatofibrosarcoma protuberans is a rare skin <span class=\"term\" data-term-id=\"289\">tumor<\/span> that arises in the deepest layer of the skin (the <span class=\"term\" data-term-id=\"61\">dermis<\/span>) It grows slowly but has a tendency to reappear after <span class=\"term\" data-term-id=\"848\">excision<\/span>. Fortunately, it rarely spreads beyond the skin.<\/p>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\"><span class=\"ez-toc-section\" id=\"Dermatofibrosarcoma-protuberans\"><\/span>Dermatofibrosarcoma protuberans <span class=\"ez-toc-section-end\"><\/span><\/h5>\n<\/section>\n<section class=\"imageLinkBlock\">\n<div class=\"flex\">\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='dfsp-1__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-1738929-4710538' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/dfsp-1__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-1738929.jpg'>\n<\/div>\n<p class=\"p-small\">Injury in 2014<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='dfsp-2__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildbd-1432115-3244039' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/dfsp-2__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDBd-1432115.jpg'>\n<\/div>\n<p class=\"p-small\">Same injury in 2015<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='dfs__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisinkildm2xq-4527398-8339820' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/dfs__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsInkiLDM2XQ-4527398.jpg'>\n<\/div>\n<p class=\"p-small\">Another example<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQue-causa-el-dermatofibrosarcoma-protuberans\"><\/span>What causes dermatofibrosarcoma protuberans?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The cause of d<span>ermatofibrosarcoma protuberans <\/span>it is unknown, but an injury to the affected skin may be a predisposing factor. Recent Advances Show Tumor Cells Are Abnormal <span class=\"term\" data-term-id=\"1272\">chromosomes<\/span> within tumor cells - t (17; 22) (q22; q13) - resulting in fusion <span class=\"term\" data-term-id=\"774\">gene<\/span> <em>COL1A1-PDGFB<\/em>. This encodes a protein that causes a tumor to grow by autocrine overproduction of <span class=\"term\" data-term-id=\"1089\">platelet<\/span>-derivative <span class=\"term\" data-term-id=\"523\">growth factor<\/span> (PDGF).<\/p>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n<div id=\"dermnet-dermnet-mobbox\">\n<\/div><\/div><\/div><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQuien-esta-en-riesgo-de-dermatofibrosarcoma-protuberans\"><\/span>Who is at risk for dermatofibrosarcoma protuberans?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p><span>re<\/span><span>ermatofibrosarcoma protuberans<\/span> it is rare and affects less than 1 person per 100,000 residents per year.<\/p>\n<ul>\n<li>It usually occurs in early to mid adult life between the ages of 20 and 59, but all ages can be affected. The tumor is rare in children.<\/li>\n<li>Males are affected slightly more often than females.<\/li>\n<li>There doesn&#039;t seem to be any race <span class=\"term\" data-term-id=\"1227\">predilection<\/span>\n<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCuales-son-los-signos-y-sintomas-del-dermatofibrosarcoma-protuberante\"><\/span>What are the signs and symptoms of dermatofibrosarcoma protruding?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p><span>re<\/span><span>ermatofibrosarcoma protuberans<\/span> usually presents as a painless thickened area of skin (<span class=\"term\" data-term-id=\"235\">license plate<\/span>) me <span class=\"term\" data-term-id=\"198\">nodule<\/span> that feels rubbery or firm to the touch and sticks to the underlying skin. It can be red-brown or skin-colored. It usually grows very slowly over months or years.<\/p>\n<p>In rare cases, it presents as a soft, depressed area of skin that makes diagnosis even more difficult. <span>re<\/span><span>ermatofibrosarcoma protuberans<\/span> it can vary in size from 0.5 to 25 cm in diameter. Fifty to sixty percent of tumors arise on the trunk, often in the shoulder and chest area. The remaining 35% of tumors are found in the extremities and 10 to 15% in the head and neck region.<\/p>\n<p><span>re<\/span><span>ermatofibrosarcoma protuberans<\/span> It is often diagnosed when it enters a more rapid growth phase that results in larger lesions. Neglected tumors can reach large proportions.<\/p>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\"><span class=\"ez-toc-section\" id=\"Mas-ejemplos-de-dermatofibrosarcoma-protuberans\"><\/span>More examples of dermatofibrosarcoma protuberans<span class=\"ez-toc-section-end\"><\/span><\/h5>\n<\/section>\n<section class=\"imageLinkBlock\">\n<div class=\"flex\">\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='dfsp__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-8389415-4988533' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/dfsp__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-8389415.jpg'>\n<\/div>\n<p class=\"p-small\">Dermatofibrosarcoma protuberans<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='dfsp-001__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-9551689-2080037' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/dfsp-001__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-9551689.jpg'>\n<\/div>\n<p class=\"p-small\">Dermatofibroscarcoma protuberans<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='dfsp-001-dermoscopy__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-5274455-4425204' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/dfsp-001-dermoscopy__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-5274455.jpg'>\n<\/div>\n<p class=\"p-small\">Dermoscopy view<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFComo-se-diagnostica-el-dermatofibrosarcoma-protuberans\"><\/span>How is dermatofibrosarcoma protuberans diagnosed?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The absence of symptoms often leads to a delay in the diagnosis of <span>re<\/span><span>ermatofibrosarcoma protuberans<\/span>. Redness and pain only occur in 15% of cases. It is often confused with other skin conditions, particularly in the early stages. Dermoscopy is not diagnostic as the characteristics of <span>re<\/span><span>ermatofibrosarcoma protuberans<\/span> are unspecific Skin <span class=\"term\" data-term-id=\"24\">biopsy<\/span> It is necessary to confirm the diagnosis.<\/p>\n<p><span>Dermatofibrosarcoma protuberans<\/span> It has a characteristic appearance under the microscope with cells densely arranged in a spindle shape. Complete removal can be difficult to assess due to extensive extensions to the skin and deeper structures. It is important to identify fibrosarcomatous <span>re<\/span><span>ermatofibrosarcoma protuberans<\/span>, a more aggressive tumor, which requires more aggressive treatment.<\/p>\n<p><span>re<\/span><span>ermatofibrosarcoma protuberans<\/span>&#039;s <span class=\"term\" data-term-id=\"1455\">chromosomal<\/span> Abnormalities can be detected using reverse transcription.<span class=\"term\" data-term-id=\"941\">Polymerase chain reaction<\/span> (RT-<span class=\"term\" data-term-id=\"942\">PCR<\/span>) or fluorescence <span class=\"term\" data-term-id=\"1077\">in the place<\/span> hybridization (<span class=\"term\" data-term-id=\"1237\">FISH<\/span>)<\/p>\n<p>In most cases of <span>re<\/span><span>ermatofibrosarcoma protuberans<\/span>, no further investigations are necessary. If there is suspicion of <span class=\"term\" data-term-id=\"185\">metastasis<\/span> or there is a fibrosarcomatous transformation, <span class=\"term\" data-term-id=\"1774\">lymph node<\/span> <span class=\"term\" data-term-id=\"1378\">ultrasound<\/span>, chest x-ray and pelvic ultrasound can be arranged.<\/p>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n<div id=\"dermnet-dermnet-mobbox\">\n<\/div><\/div><\/div><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCual-es-el-tratamiento-para-dermatofibrosarcoma-protuberans\"><\/span>What is the treatment for dermatofibrosarcoma protuberans?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Treatment of <span>re<\/span><span>ermatofibrosarcoma protuberans<\/span> and <span>re<\/span><span>ermatofibrosarcoma protuberans<\/span> with fibrosarcomatous transformation consists of <span class=\"term\" data-term-id=\"1798\">wide excision<\/span> of the <span class=\"term\" data-term-id=\"164\">injury<\/span> including deep <span class=\"term\" data-term-id=\"1761\">fascia<\/span>, with 1\u20133 cm margin of normal skin. It may take more than one surgical procedure to ensure complete removal of a tumor.<\/p>\n<p>Mohs micrographic surgery, which is a special surgical technique for <span class=\"term\" data-term-id=\"1854\">control<\/span> tumor margins, sometimes used to verify that all abnormal cells have been removed. <span class=\"term\" data-term-id=\"1074\">Reappearance<\/span> after Mohs surgery it is reported to be around 1%.<\/p>\n<p><span class=\"term\" data-term-id=\"1325\">Radiotherapy<\/span> it is sometimes used in addition to surgery if a tumor cannot be completely removed by surgery. <span class=\"term\" data-term-id=\"1619\">Chemotherapy<\/span> It is ineffective.<\/p>\n<p>the <span class=\"term\" data-term-id=\"624\">tyrosine kinase<\/span> The inhibitory imatinib mesylate is used to treat rare cases of local advanced inoperable or <span class=\"term\" data-term-id=\"1000\">metastatic<\/span> dermatofibrosarcoma characterized by <em>COL1A1-PDGFB<\/em> fusion gene, with 50% response rates.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"Cual-es-el-pronostico-para-dermatofibrosarcoma-protuberans\"><\/span>Which is the <span class=\"term\" data-term-id=\"241\">forecast<\/span> for dermatofibrosarcoma protuberans?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Follow-up with clinical examination of the <span>re<\/span><span>ermatofibrosarcoma protuberans<\/span> It is recommended every 6 months for 5 years, and then annually.<\/p>\n<p>The tumor only metastasizes in 5% of cases. It spreads on 1% via <span class=\"term\" data-term-id=\"744\">lymphatic<\/span> vessels to the regional lymphatic glands and at 4% through the bloodstream, most commonly to the lung followed by the brain, bones, and heart.<\/p>\n<p>Local recurrences arise in 11-20% of cases, generally within 3 years of the initial surgery, so follow-up is important. Recurrences are treated surgically as described for the original. <span class=\"term\" data-term-id=\"949\">primary<\/span> tumor.<\/p>\n<\/section>","protected":false},"excerpt":{"rendered":"<p>\u00bfQu\u00e9 es el dermatofibrosarcoma protuberante? Dermatofibrosarcoma protuberans es una piel poco com\u00fan tumor que surge en la capa m\u00e1s profunda de la piel (la dermis) Crece lentamente pero tiene una&#8230;<\/p>","protected":false},"author":8,"featured_media":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","format":"standard","meta":{"footnotes":""},"categories":[204],"tags":[],"class_list":["post-7858","wiki","type-wiki","status-publish","format-standard","category-glosario-definiciones"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/7858","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki"}],"about":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/types\/wiki"}],"author":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/comments?post=7858"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/7858\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/media?parent=7858"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/categories?post=7858"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/tags?post=7858"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}