{"id":2393,"date":"2022-01-28T22:34:55","date_gmt":"2022-01-29T01:34:55","guid":{"rendered":"https:\/\/gastropedia.pub\/pt\/tumores-neuroendocrinos-do-pancreas\/"},"modified":"2023-08-26T19:42:42","modified_gmt":"2023-08-26T22:42:42","slug":"tumores-neuroendocrinos-do-pancreas","status":"publish","type":"post","link":"https:\/\/gastropedia.pub\/pt\/cirurgia\/hepatopancreatobiliar\/tumores-neuroendocrinos-do-pancreas\/","title":{"rendered":"Tumores neuroend\u00f3crinos do pancreas"},"content":{"rendered":"<div class=\"pdfprnt-buttons pdfprnt-buttons-post pdfprnt-top-right\"><a href=\"https:\/\/gastropedia.pub\/pt\/wp-json\/wp\/v2\/posts\/2393?print=pdf\" class=\"pdfprnt-button pdfprnt-button-pdf\" target=\"_blank\" ><img decoding=\"async\" src=\"https:\/\/gastropedia.pub\/pt\/wp-content\/plugins\/pdf-print\/images\/pdf.png\" alt=\"image_pdf\" title=\"Ver PDF\" \/><\/a><a href=\"https:\/\/gastropedia.pub\/pt\/wp-json\/wp\/v2\/posts\/2393?print=print\" class=\"pdfprnt-button pdfprnt-button-print\" target=\"_blank\" ><img decoding=\"async\" src=\"https:\/\/gastropedia.pub\/pt\/wp-content\/plugins\/pdf-print\/images\/print.png\" alt=\"image_print\" title=\"Conte\u00fado de impress\u00e3o\" \/><\/a><\/div>\n<h2 class=\"wp-block-heading\" id=\"h-introducao\"><strong>Introdu\u00e7\u00e3o<\/strong><\/h2>\n\n\n\n<p>A incid\u00eancia dos tumores neuroend\u00f3crinos do p\u00e2ncreas est\u00e1 crescendo, possivelmente devido \u00e0 realiza\u00e7\u00e3o com maior frequ\u00eancia exames de imagem e \u00e0 qualidade destes exames. No entanto, sua preval\u00eancia felizmente ainda \u00e9 rara. Esse post do Endoscopia Terap\u00eautica tem a finalidade de servir como um guia de consulta quando eventualmente nos depararmos com uma dessas situa\u00e7\u00f5es no dia a dia. Se quiser saber sobre <a href=\"https:\/\/gastropedia.pub\/pt\/gastroenterologia\/intestino\/atualizacao-em-tnes-gastricos-e-duodenais-tudo-o-que-voce-precisa-saber\/\">tumores neuroend\u00f3crinos duodenais confira esse outro artigo<\/a>.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\" id=\"h-conceitos-gerais-importantes-sobre-tumores-neuroendocrinos-do-trato-gastrointestinal\"><strong>Conceitos gerais importantes sobre tumores neuroend\u00f3crinos do trato gastrointestinal<\/strong><\/h2>\n\n\n\n<p>Os TNE correspondem a um grupo heterog\u00eaneo de neoplasias que se originam de c\u00e9lulas neuroend\u00f3crinas (c\u00e9lulas enterocromafins-like), com caracter\u00edsticas secret\u00f3rias.<\/p>\n\n\n\n<p>Todos os TNE gastroenteropancre\u00e1ticos (GEP) s\u00e3o potencialmente malignos e o comportamento e progn\u00f3stico est\u00e3o correlacionados com os tipos histol\u00f3gicos.<\/p>\n\n\n\n<p>Os TNE podem ser <strong>espor\u00e1dicos (90%)<\/strong> ou <strong>associados a s\u00edndromes heredit\u00e1rias (10%)<\/strong>, como a neoplasia end\u00f3crina m\u00faltipla tipo 1 (NEM-1), SD von Hippel-Lindau, neurofibromatose e esclerose tuberosa.<\/p>\n\n\n\n<p>Os TNE s\u00e3o na sua maioria indolentes, mas podem determinar sintomas. Desta forma, podem ser divididos em funcionantes e n\u00e3o funcionantes:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Funcionantes:<\/strong> secre\u00e7\u00e3o de horm\u00f4nios ou neurotransmissores ativos: serotonina, glucagon, insulina, somatostatina, gastrina, histamina, VIP ou catecolaminas. Podem causar uma variedade de sintomas<\/li>\n\n\n\n<li><strong>N\u00e3o funcionantes:<\/strong> podem n\u00e3o secretar nenhum pept\u00eddeo\/ horm\u00f4nios ou secretar pept\u00eddeos ou neurotransmissores n\u00e3o ativos, de forma a n\u00e3o causar manifesta\u00e7\u00f5es cl\u00ednicas.<\/li>\n<\/ul>\n\n\n\n<h2 class=\"wp-block-heading\" id=\"h-tumores-neuroendocrinos-do-pancreas-tne-p\"><strong>Tumores neuroend\u00f3crinos do p\u00e2ncreas (TNE-P)<\/strong><\/h2>\n\n\n\n<p>Os TNE funcionantes do p\u00e2ncreas s\u00e3o: insulinoma, gastrinoma, glucagonoma, vipoma e somatostatinoma.<\/p>\n\n\n\n<p>Maioria dos&nbsp;TNE-P s\u00e3o malignos, exce\u00e7\u00e3o aos insulinomas e TNE-NF menores que 2 cm.<\/p>\n\n\n\n<p>A cirurgia \u00e9 a \u00fanica modalidade curativa para TNE-P espor\u00e1dicos, e a ressec\u00e7\u00e3o do tumor prim\u00e1rio em pacientes com doen\u00e7a localizada, regional e at\u00e9 metast\u00e1tica, pode melhorar a sobrevida do paciente.<\/p>\n\n\n\n<p>De maneira geral, TNE funcionantes do pancreas devem ser ressecados para controle dos sintomas sempre que poss\u00edvel. TNE-NF depende do tamanho (ver abaixo).<\/p>\n\n\n\n<p>Tumores pancre\u00e1ticos m\u00faltiplos s\u00e3o raros e devem despertar a suspeita de NEM1.<\/p>\n\n\n\n<p class=\"has-text-align-center has-background\" style=\"background-color:#abb7c27d\"><strong>A SEGUIR VAMOS VER AS PRINCIPAIS CARACTER\u00cdSTICAS DE CADA SUBTIPO HISTOL\u00d3GICO<\/strong><\/p>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-insulinomas\"><strong>INSULINOMAS<\/strong><\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li>\u00c9 o TNE mais frequente das ilhotas pancre\u00e1ticas.<\/li>\n\n\n\n<li>90% s\u00e3o benignos, por\u00e9m s\u00e3o sintom\u00e1ticos mesmo quando pequenos.<\/li>\n\n\n\n<li>Cerca de 10% est\u00e3o associadas a NEM.<\/li>\n\n\n\n<li>S\u00e3o les\u00f5es hipervascularizadas e solit\u00e1rias, frequentemente &lt; 2 cm.<\/li>\n\n\n\n<li><strong>Tr\u00edade de Whipple:<\/strong>\n<ul class=\"wp-block-list\">\n<li>hipoglicemia (&lt; 50)<\/li>\n\n\n\n<li>sintomas neuroglicopenicos (borramento visual, fraqueza, cansa\u00e7o, cefaleia, sonol\u00eancia)<\/li>\n\n\n\n<li>desaparecimento dos sintomas com a reposi\u00e7\u00e3o de glicose<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li>insulina s\u00e9rica &gt; 6 UI\/ml<\/li>\n\n\n\n<li>Pept\u00eddeo C &gt; 0,2 mmol\/l<\/li>\n\n\n\n<li>Pr\u00f3-insulina &gt; 5 UI\/ml<\/li>\n\n\n\n<li>Teste de jejum prolongado positivo (99% dos casos)<\/li>\n\n\n\n<li><a href=\"https:\/\/gastropedia.pub\/pt\/cirurgia\/insulinoma-estrategias-diagnosticas-e-detalhes-para-o-tratamento\">Saiba mais sobre insulinoma nesse outro artigo<\/a><\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-gastrinomas\"><strong>GASTRINOMAS<\/strong><\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li>\u00c9 mais comum no duodeno, mas 30% dos casos est\u00e3o no p\u00e2ncreas<\/li>\n\n\n\n<li>S\u00e3o os TNE do p\u00e2ncreas mais frequentes depois dos insulinomas.<\/li>\n\n\n\n<li>Est\u00e3o associados a Sd. NEM 1 em 30%, e nesses casos se apresentam como les\u00f5es pequenas e multifocais.<\/li>\n\n\n\n<li>Provocam hipergastrinemia e s\u00edndrome de Zollinger-Ellison.<\/li>\n\n\n\n<li>60% s\u00e3o malignos.<\/li>\n\n\n\n<li>Tratamento: cir\u00fargico nos espor\u00e1dicos (DPT).<\/li>\n\n\n\n<li>Na NEM 1, h\u00e1 controv\u00e9rsia na indica\u00e7\u00e3o cir\u00fargica, visto que pode n\u00e3o haver o controle da hipergastrinemia mesmo com a DPT (tumores costumam ser m\u00faltiplos)<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-glucagonomas\"><strong>GLUCAGONOMAS<\/strong><\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Raros;&nbsp;&nbsp;maioria&nbsp;espor\u00e1dicos.<\/li>\n\n\n\n<li>Geralmente, s\u00e3o grandes e solit\u00e1rios, com tamanho entre 3-7 cm ocorrendo principalmente na cauda do p\u00e2ncreas.<\/li>\n\n\n\n<li>Sintomas: eritema&nbsp;necrol\u00edtico&nbsp;migrat\u00f3rio (80%), DM, desnutri\u00e7\u00e3o, perda de peso, tromboflebite, glossite, queilite angular, anemia<\/li>\n\n\n\n<li>Crescimento lento e sobrevida longa<\/li>\n\n\n\n<li>Met\u00e1stase linfonodal ou hep\u00e1tica ocorre em 60-75% dos casos.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-vipomas\"><strong>VIPOMAS<\/strong><\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Extremamente raros<\/li>\n\n\n\n<li>Como os glucagonomas, localizados na cauda, grandes e solit\u00e1rios.<\/li>\n\n\n\n<li>Maioria maligno e metast\u00e1tico<\/li>\n\n\n\n<li>Em 10% dos casos pode ser extra-pancre\u00e1tico.<\/li>\n\n\n\n<li>Quadro cl\u00ednico&nbsp;relacionada a secre\u00e7\u00e3o do VIP (pept\u00eddeo vasoativo intestinal):\n<ul class=\"wp-block-list\">\n<li>diarr\u00e9ia&nbsp;(mais de 3L litros por dia) \u2013 \u00e1gua de lavado de arroz<\/li>\n\n\n\n<li>Dist\u00farbios hidro-eletrol\u00edtico: hipocalemia, hipocloridria, acidose metab\u00f3lica<\/li>\n\n\n\n<li>Rubor<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li>Excelente resposta ao tratamento com an\u00e1logos da somatostatina.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-somatostatinomas\"><strong>SOMATOSTATINOMAS<\/strong><\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li>\u00c9 o menos comum de todos<\/li>\n\n\n\n<li>Somatostatina leva&nbsp;a&nbsp;inibi\u00e7\u00e3o da secre\u00e7\u00e3o end\u00f3crina e ex\u00f3crina e afeta a motilidade intestinal.<\/li>\n\n\n\n<li>Les\u00e3o&nbsp;solit\u00e1ria, grande, espor\u00e1dico, maioria maligno e metastatico<\/li>\n\n\n\n<li>Quadro cl\u00ednico:\n<ul class=\"wp-block-list\">\n<li>Diabetes (75%)<\/li>\n\n\n\n<li>Colelit\u00edase (60%)<\/li>\n\n\n\n<li>Esteatorr\u00e9ia (60%)<\/li>\n\n\n\n<li>Perda de peso<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-tne-nao-funcionantes-do-pancreas\"><strong>TNE N\u00c3O FUNCIONANTES DO P\u00c2NCREAS<\/strong><\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li>20% de todos os TNEs do p\u00e2ncreas.<\/li>\n\n\n\n<li>50% s\u00e3o malignos.<\/li>\n\n\n\n<li><strong>O principal diagn\u00f3stico diferencial \u00e9 com o adenocarcinoma<\/strong><\/li>\n<\/ul>\n\n\n\n<p class=\"has-background has-normal-font-size\" style=\"background-color:#8dd2fc29\"><em><strong>TNE-NF bem diferenciados menores que 2 cm: <\/strong>duas sociedades (ENETS e NCCN) sugerem observa\u00e7\u00e3o se for bem diferenciado. Entretanto, a sociedade norte-americana NETS recomenda observa\u00e7\u00e3o em tumores <strong>menores que 1 cm<\/strong> e <u>conduta individualizada<\/u>, entre 1-2 cm.<\/em><\/p>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-tne-pancreatico-relacionados-a-sindromes-hereditarias\"><strong>TNE PANCRE\u00c1TICO RELACIONADOS A SINDROMES HEREDITARIAS<\/strong><\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li>10% dos TNE-P s\u00e3o relacionados a NEM-1<\/li>\n\n\n\n<li>Frequentemente <strong>multic\u00eantricos<\/strong>,<\/li>\n\n\n\n<li>Geralmente acometendo pessoas mais jovens.<\/li>\n\n\n\n<li>Geralmente de comportamento benigno, por\u00e9m apresentam potencial maligno<\/li>\n\n\n\n<li>Gastrinoma 30-40%; Insulinoma 10%; TNE-NF 20-50%; outros 2%<\/li>\n\n\n\n<li>Tto cir\u00fargico \u00e9 controverso, pq as vezes n\u00e3o controla a gastrinemia (tumores m\u00faltiplos)<\/li>\n<\/ul>\n\n\n\n<figure class=\"wp-block-pullquote has-small-font-size\" style=\"font-style:normal;font-weight:300\"><blockquote><p><em>Voc\u00ea se recorda das neoplasias neuroend\u00f3crinas m\u00faltiplas?&nbsp;<\/em><\/p><\/blockquote><\/figure>\n\n\n\n<p>As s\u00edndromes de neoplasia end\u00f3crina m\u00faltipla (NEM) compreendem 3 doen\u00e7as familiares geneticamente distintas envolvendo hiperplasia adenomatosa e tumores malignos em v\u00e1rias gl\u00e2ndulas end\u00f3crinas. S\u00e3o doen\u00e7as autoss\u00f4micas dominantes.<\/p>\n\n\n\n<h5 class=\"wp-block-heading\" id=\"h-nem-1\">NEM-1<\/h5>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Doen\u00e7a autoss\u00f4mica dominante<\/li>\n\n\n\n<li>Predispoe a TU (3Ps): Paratire\u00f3ide; Pituit\u00e1ria (hip\u00f3fise); P\u00e2ncreas,<\/li>\n\n\n\n<li>Geralmente de comportamento benigno, por\u00e9m apresentam potencial maligno<\/li>\n\n\n\n<li>Gastrinoma 30-40%; Insulinoma 10%; TNE-NF 20-50%; outros 2%<\/li>\n\n\n\n<li>Tto cir\u00fargico \u00e9 controverso, pq as vezes n\u00e3o controla a gastrinemia (tumores m\u00faltiplos)<\/li>\n<\/ul>\n\n\n\n<h5 class=\"wp-block-heading\" id=\"h-nem-2a\">NEM-2A:<\/h5>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Carcinoma medular da tireoide,<\/li>\n\n\n\n<li>Feocromocitoma,<\/li>\n\n\n\n<li>Hiperplasia ou adenomas das gl\u00e2ndulas paratireoides (com consequente hiperparatireoidismo).<\/li>\n<\/ul>\n\n\n\n<h5 class=\"wp-block-heading\" id=\"h-nem-2b\">NEM-2B:<\/h5>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Carcinoma medular de tireoide,<\/li>\n\n\n\n<li>Feocromocitoma<\/li>\n\n\n\n<li>Neuromas mucosos e intestinais m\u00faltiplos<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-referencias\"><strong>Refer\u00eancias:<\/strong><\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li>Pathology, classification, and grading of neuroendocrine neoplasms arising in the digestive system &#8211; UpToDate<span class=\"Apple-converted-space\"> ; 2021<\/span><\/li>\n\n\n\n<li>Guidelines for the management of neuroendocrine tumours by the Brazilian gastrointestinal tumour group. ecancer 2017,11:716 DOI: 10.3332\/ecancer.2017.716<\/li>\n<\/ol>\n\n\n\n<h3 class=\"wp-block-heading\" id=\"h-como-citar-esse-artigo\">Como citar esse artigo:<\/h3>\n\n\n\n<p class=\"has-background\" style=\"background-color:#abb7c282\">Martins BC, de Moura DTH. Tumores neuroend\u00f3crinos do pancreas. Gasstropedia. 2022; vol I.&nbsp; Dispon\u00edvel em: gastropedia.pub\/pt\/cirurgia\/tumores-neuroendocrinos-do-pancreas<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Introdu\u00e7\u00e3o A incid\u00eancia dos tumores neuroend\u00f3crinos do p\u00e2ncreas est\u00e1 crescendo, possivelmente devido \u00e0 realiza\u00e7\u00e3o com maior frequ\u00eancia exames de imagem e \u00e0 qualidade destes exames. No entanto, sua preval\u00eancia felizmente&hellip;<\/p>\n","protected":false},"author":722,"featured_media":2394,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"","_lmt_disable":"","footnotes":""},"categories":[24,18,28,23],"tags":[67,68,69,70,71,72],"ano":[43],"tipo":[121],"volume":[57],"class_list":["post-2393","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-cirurgia","category-gastroenterologia","category-hepatopancreatobiliar","category-pancreas","tag-glucagonoma","tag-insulinoma","tag-nem","tag-nem-1","tag-tumor-neuroendocrino","tag-vipoma","ano-43","tipo-assuntos-gerais","volume-volume-i"],"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v27.2 (Yoast SEO v27.3) - https:\/\/yoast.com\/product\/yoast-seo-premium-wordpress\/ -->\n<title>Tumores neuroend\u00f3crinos do pancreas - Gastropedia<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/gastropedia.pub\/pt\/cirurgia\/hepatopancreatobiliar\/tumores-neuroendocrinos-do-pancreas\/\" \/>\n<meta property=\"og:locale\" content=\"pt_BR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Tumores neuroend\u00f3crinos do pancreas\" \/>\n<meta property=\"og:description\" content=\"Introdu\u00e7\u00e3o A incid\u00eancia dos tumores neuroend\u00f3crinos do p\u00e2ncreas est\u00e1 crescendo, possivelmente devido \u00e0 realiza\u00e7\u00e3o com maior frequ\u00eancia exames de imagem e \u00e0 qualidade destes exames. 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