{"id":8287,"date":"2020-04-28T00:49:14","date_gmt":"2020-04-28T03:49:14","guid":{"rendered":"https:\/\/doctorhoogstra.com\/es\/wiki\/leiomiomatosis-hereditaria-y-sindrome-de-cancer-de-celulas-renales\/"},"modified":"2020-10-30T07:40:49","modified_gmt":"2020-10-30T10:40:49","slug":"sindrome-de-cancer-hereditario-leiomiomatose-e-celula-renal","status":"publish","type":"wiki","link":"https:\/\/doctorhoogstra.com\/pt\/wiki\/leiomiomatosis-hereditaria-y-sindrome-de-cancer-de-celulas-renales\/","title":{"rendered":"Leiomiomatose heredit\u00e1ria e s\u00edndrome do c\u00e2ncer de c\u00e9lulas renais."},"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\">Conte\u00fado<\/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=\"Alternar tabela de conte\u00fado\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Alternar<\/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\/pt\/wiki\/leiomiomatosis-hereditaria-y-sindrome-de-cancer-de-celulas-renales\/#Que-es-hereditario-leiomiomatosis-y-renal-celula-cancer-sindrome\" >O que \u00e9 leiomiomatose heredit\u00e1ria e s\u00edndrome do c\u00e2ncer de c\u00e9lulas renais?<\/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\/pt\/wiki\/leiomiomatosis-hereditaria-y-sindrome-de-cancer-de-celulas-renales\/#Leiomiomatosis-cutanea\" >Leiomiomatose cut\u00e2nea<\/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\/pt\/wiki\/leiomiomatosis-hereditaria-y-sindrome-de-cancer-de-celulas-renales\/#%C2%BFCuales-son-las-caracteristicas-clinicas-de-la-leiomiomatosis-hereditaria-y-el-sindrome-de-cancer-de-celulas-renales\" >Quais s\u00e3o as caracter\u00edsticas cl\u00ednicas da leiomiomatose heredit\u00e1ria e da s\u00edndrome do c\u00e2ncer de c\u00e9lulas renais?<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/doctorhoogstra.com\/pt\/wiki\/leiomiomatosis-hereditaria-y-sindrome-de-cancer-de-celulas-renales\/#Leiomioma-cutaneo\" >leiomioma cut\u00e2neo<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/doctorhoogstra.com\/pt\/wiki\/leiomiomatosis-hereditaria-y-sindrome-de-cancer-de-celulas-renales\/#Leiomioma-uterino\" >leiomioma uterino<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/doctorhoogstra.com\/pt\/wiki\/leiomiomatosis-hereditaria-y-sindrome-de-cancer-de-celulas-renales\/#Carcinoma-de-celulas-renales\" >Carcinoma de c\u00e9lulas renais<\/a><\/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\/pt\/wiki\/leiomiomatosis-hereditaria-y-sindrome-de-cancer-de-celulas-renales\/#%C2%BFCual-es-la-causa-de-la-leiomiomatosis-hereditaria-y-el-sindrome-de-cancer-de-celulas-renales\" >Qual \u00e9 a causa da leiomiomatose heredit\u00e1ria e da s\u00edndrome do c\u00e2ncer de c\u00e9lulas renais?<\/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\/pt\/wiki\/leiomiomatosis-hereditaria-y-sindrome-de-cancer-de-celulas-renales\/#%C2%BFCual-es-el-tratamiento-para-el-sindrome\" >Qual \u00e9 o tratamento para a s\u00edndrome?<\/a><\/li><\/ul><\/nav><\/div>\n\n<section class=\"textBlock\">\n<h2><span class=\"ez-toc-section\" id=\"Que-es-hereditario-leiomiomatosis-y-renal-celula-cancer-sindrome\"><\/span>O que \u00e9 <span class=\"term\" data-term-id=\"1745\">heredit\u00e1rio<\/span> leiomiomatose e <span class=\"term\" data-term-id=\"896\">renal<\/span> c\u00e9lula <span class=\"term\" data-term-id=\"30\">C\u00e2ncer<\/span> <span class=\"term\" data-term-id=\"350\">s\u00edndrome<\/span>?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>A leiomiomatose heredit\u00e1ria e a s\u00edndrome do c\u00e2ncer de c\u00e9lulas renais s\u00e3o uma condi\u00e7\u00e3o rara que foi formalmente reconhecida pela primeira vez em 2001. Atualmente, \u00e9 relatado que afeta mais de 105 fam\u00edlias em todo o mundo. As caracter\u00edsticas da s\u00edndrome incluem:<\/p>\n<ul>\n<li><span class=\"term\" data-term-id=\"47\">Cut\u00e2neo<\/span> leiomiomas<\/li>\n<li>Leiomiomas uterinos<\/li>\n<li>Uterino <span class=\"term\" data-term-id=\"1673\">leiomiossarcoma<\/span>\n<\/li>\n<li>C\u00e2nceres de C\u00e9lulas Renais<\/li>\n<\/ul>\n<p><span>A leiomiomatose heredit\u00e1ria e a s\u00edndrome do c\u00e2ncer de c\u00e9lulas renais tamb\u00e9m s\u00e3o conhecidas como s\u00edndrome de Reed, leiomiomas m\u00faltiplos cut\u00e2neos e uterinos tipo 1 e leiomiomas heredit\u00e1rios de c\u00e9lulas renais. <span class=\"term\" data-term-id=\"32\">carcinoma<\/span>.<\/span><\/p>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\"><span class=\"ez-toc-section\" id=\"Leiomiomatosis-cutanea\"><\/span>Leiomiomatose cut\u00e2nea<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='leiomiomatose-4__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingilde5xq-9771940-6311204' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/leiomyomatosis-4__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDE5XQ-9771940.jpg'>\n<\/div>\n<p class=\"p-small\">Leiomiomatose cut\u00e2nea<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='leiomiomatose-3__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisinkildm0xq-5297460-5309038' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/leiomyomatosis-3__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsInkiLDM0XQ-5297460.jpg'>\n<\/div>\n<p class=\"p-small\">Leiomiomatose cut\u00e2nea<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='leiomiomatose-2__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildizxq-5191163-7532360' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/leiomyomatosis-2__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDIzXQ-5191163.jpg'>\n<\/div>\n<p class=\"p-small\">Leiomiomatose cut\u00e2nea<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCuales-son-las-caracteristicas-clinicas-de-la-leiomiomatosis-hereditaria-y-el-sindrome-de-cancer-de-celulas-renales\"><\/span>Quais s\u00e3o as caracter\u00edsticas cl\u00ednicas da leiomiomatose heredit\u00e1ria e da s\u00edndrome do c\u00e2ncer de c\u00e9lulas renais?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<h3><span class=\"ez-toc-section\" id=\"Leiomioma-cutaneo\"><\/span>leiomioma cut\u00e2neo<span class=\"ez-toc-section-end\"><\/span><\/h3>\n<p>Leiomiomas cut\u00e2neos ou piloleiomiomas aparecem como pele firme pequena (0,5 a 2 cm) de cor firme <span class=\"term\" data-term-id=\"367\">n\u00f3dulos<\/span>. surgem do <span class=\"term\" data-term-id=\"15\">m\u00fasculos eretores do pelo<\/span> eles s\u00e3o respons\u00e1veis por deixar seu cabelo em p\u00e9. Les\u00f5es m\u00faltiplas podem se desenvolver, muitas vezes em grupos e geralmente em um <span class=\"term\" data-term-id=\"619\">segmento<\/span> <span class=\"term\" data-term-id=\"69\">distribui\u00e7\u00e3o<\/span>. Esses n\u00f3dulos s\u00e3o <span class=\"term\" data-term-id=\"22\">benigno<\/span>, ent\u00e3o eles s\u00e3o apenas uma preocupa\u00e7\u00e3o por causa de sua apar\u00eancia ou tend\u00eancia a serem sens\u00edveis se esbarrados.<\/p>\n<p>Ocasionalmente, leiomiomas cut\u00e2neos m\u00faltiplos tamb\u00e9m podem surgir em associa\u00e7\u00e3o com a s\u00edndrome de Gardner, <span class=\"term\" data-term-id=\"127\">HIV<\/span> <span class=\"term\" data-term-id=\"145\">infec\u00e7\u00e3o<\/span>, <span class=\"term\" data-term-id=\"319\">cr\u00f4nica<\/span> <span class=\"term\" data-term-id=\"422\">linfoc\u00edtico<\/span> <span class=\"term\" data-term-id=\"613\">leucemia<\/span> e <span class=\"term\" data-term-id=\"1457\">policitemia<\/span>.<\/p>\n<h3><span class=\"ez-toc-section\" id=\"Leiomioma-uterino\"><\/span>leiomioma uterino<span class=\"ez-toc-section-end\"><\/span><\/h3>\n<p>Ele <a class=\"wpil_keyword_link\" href=\"https:\/\/drhoogstra.com.ar\/wiki\/leiomioma\/\" title=\"leiomioma\">leiomioma<\/a> \u00fatero tamb\u00e9m \u00e9 conhecido como mioma e \u00e9 o mais comum na pr\u00e9-menopausa. <span class=\"term\" data-term-id=\"289\">tumor<\/span> em mulheres Na leiomiomatose heredit\u00e1ria e na s\u00edndrome do c\u00e2ncer de c\u00e9lulas renais, os miomas ocorrem mais cedo do que o normal e geralmente s\u00e3o maiores e t\u00eam maior probabilidade de causar sangramento excessivo e dor durante a menstrua\u00e7\u00e3o. Embora a maioria seja benigna, existe um risco aumentado de <span class=\"term\" data-term-id=\"1742\">malignidade<\/span> (leiomiossarcoma) em desenvolvimento, e alguns aparecem <span class=\"term\" data-term-id=\"1395\">at\u00edpico<\/span> atrav\u00e9s do microsc\u00f3pio<\/p>\n<h3><span class=\"ez-toc-section\" id=\"Carcinoma-de-celulas-renales\"><\/span>Carcinoma de c\u00e9lulas renais<span class=\"ez-toc-section-end\"><\/span><\/h3>\n<p>O carcinoma de c\u00e9lulas renais \u00e9 a caracter\u00edstica mais preocupante da leiomiomatose heredit\u00e1ria e da s\u00edndrome do c\u00e2ncer de c\u00e9lulas renais, embora isso n\u00e3o ocorra em todas as pessoas com a doen\u00e7a. Acredita-se que apenas alguns <span class=\"term\" data-term-id=\"871\">muta\u00e7\u00f5es<\/span> est\u00e3o associados ao desenvolvimento de c\u00e2ncer de c\u00e9lulas renais. Infelizmente, os c\u00e2nceres de c\u00e9lulas renais que se desenvolvem nessa s\u00edndrome tendem a ser agressivos e aparecem em uma idade jovem. Eles s\u00e3o <span class=\"term\" data-term-id=\"1669\">histologicamente<\/span> papilar tipo 2. Ao contr\u00e1rio de outras s\u00edndromes de c\u00e2ncer de c\u00e9lulas renais (ver s\u00edndrome de von Hippel Lindau e s\u00edndrome de Birt-Hogg-Dub\u00e9), elas s\u00e3o mais comuns <span class=\"term\" data-term-id=\"292\">unilateral<\/span> e solit\u00e1rio<\/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-la-causa-de-la-leiomiomatosis-hereditaria-y-el-sindrome-de-cancer-de-celulas-renales\"><\/span>Qual \u00e9 a causa da leiomiomatose heredit\u00e1ria e da s\u00edndrome do c\u00e2ncer de c\u00e9lulas renais?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>a <span class=\"term\" data-term-id=\"774\">gene<\/span> <span>respons\u00e1vel pela leiomiomatose heredit\u00e1ria e s\u00edndrome do c\u00e2ncer de c\u00e9lulas renais <\/span>foi identificado como o gene da fumarato hidratase. Acredita-se que este gene atue como um supressor de tumor. fumarato hidratase <span class=\"term\" data-term-id=\"1525\">enzima<\/span> ele funciona na via de energia celular chamada ciclo de Krebs. Como isso causa as caracter\u00edsticas cl\u00ednicas n\u00e3o \u00e9 totalmente compreendido.<\/p>\n<p>Muitas muta\u00e7\u00f5es neste gene s\u00e3o conhecidas, todas as quais resultam na s\u00edndrome sendo herdada em um <span class=\"term\" data-term-id=\"828\">autoss\u00f4mico<\/span> moda dominante. Isso significa que um indiv\u00edduo afetado pode transmiti-lo a cerca de metade de seus filhos e filhas. A consulta com um geneticista pode ser aconselh\u00e1vel dada a import\u00e2ncia do envolvimento de outros membros da fam\u00edlia.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCual-es-el-tratamiento-para-el-sindrome\"><\/span>Qual \u00e9 o tratamento para a s\u00edndrome?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>N\u00e3o h\u00e1 meios de reparar o gene. <span class=\"term\" data-term-id=\"870\">muta\u00e7\u00e3o<\/span>. O tratamento se concentra na detec\u00e7\u00e3o e preven\u00e7\u00e3o do c\u00e2ncer.<\/p>\n<p>O conselho atual \u00e9 obter imagens regulares dos rins. Tem sido sugerido que os tumores renais nesta s\u00edndrome n\u00e3o s\u00e3o vistos precocemente. <span class=\"term\" data-term-id=\"1378\">ultrassom<\/span>, ent\u00e3o uma <span class=\"term\" data-term-id=\"1116\">Resson\u00e2ncia magn\u00e9tica<\/span> digitaliza\u00e7\u00e3o \u00e9 geralmente necess\u00e1ria.<\/p>\n<p>As mulheres afetadas devem consultar um ginecologista, especialmente se tiverem sangramento intenso ou dor durante a menstrua\u00e7\u00e3o. Se eles tiverem um mioma grande, pode ser necess\u00e1rio remov\u00ea-lo por motivos sintom\u00e1ticos. A triagem uterina regular deve ser incentivada. Depois de terem filhos, eles podem querer considerar a histerectomia (remo\u00e7\u00e3o do \u00fatero) para reduzir o risco de c\u00e2ncer uterino.<\/p>\n<p>Uma vez que a leiomiomatose heredit\u00e1ria e a s\u00edndrome do cancro das c\u00e9lulas renais s\u00e3o heredit\u00e1rias, \u00e9 fundamental que todos os membros da fam\u00edlia sejam informados e consultados por um m\u00e9dico com experi\u00eancia nesta \u00e1rea. <span class=\"term\" data-term-id=\"558\">Gen\u00e9tico<\/span> testes est\u00e3o dispon\u00edveis com um simples <span class=\"term\" data-term-id=\"1296\">DNA<\/span> amostra (sangue ou swab bucal).<\/p>\n<p>Alguns casos podem exigir tratamento para dor de leiomiomas cut\u00e2neos. O seguinte foi relatado como \u00fatil:<\/p>\n<ul>\n<li>Bloqueadores dos canais de c\u00e1lcio, particularmente nifedipina.<\/li>\n<li>Um bloqueador alfa-adrenoceptor, fenoxibenzamina<\/li>\n<li>Gabapentina<\/li>\n<\/ul>\n<\/section>","protected":false},"excerpt":{"rendered":"<p>Que es hereditario leiomiomatosis y renal c\u00e9lula c\u00e1ncer s\u00edndrome? La leiomiomatosis hereditaria y el s\u00edndrome de c\u00e1ncer de c\u00e9lulas renales es una condici\u00f3n rara que se reconoci\u00f3 formalmente por primera&#8230;<\/p>","protected":false},"author":8,"featured_media":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","format":"standard","meta":{"footnotes":""},"categories":[],"tags":[],"class_list":["post-8287","wiki","type-wiki","status-publish","format-standard"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/wiki\/8287","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/wiki"}],"about":[{"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/types\/wiki"}],"author":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/comments?post=8287"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/wiki\/8287\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/media?parent=8287"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/categories?post=8287"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/tags?post=8287"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}