{"id":10908,"date":"2022-01-28T22:34:55","date_gmt":"2022-01-28T22:34:55","guid":{"rendered":"https:\/\/endoscopiaterapeutica.net\/pt\/?p=10908"},"modified":"2022-06-23T19:46:09","modified_gmt":"2022-06-23T19:46:09","slug":"tumores-neuroendocrinos-do-pancreas","status":"publish","type":"post","link":"https:\/\/endoscopiaterapeutica.net\/pt\/assuntosgerais\/tumores-neuroendocrinos-do-pancreas\/","title":{"rendered":"Tumores neuroend\u00f3crinos do pancreas"},"content":{"rendered":"

Introdu\u00e7\u00e3o<\/strong><\/h2>\n

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.<\/p>\n

Conceitos gerais importantes sobre tumores neuroend\u00f3crinos do trato gastrointestinal<\/strong><\/h2>\n

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

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

Os TNE podem ser espor\u00e1dicos (90%)<\/strong> ou 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

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
  • 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\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

    Tumores neuroend\u00f3crinos do p\u00e2ncreas (TNE-P)<\/strong><\/h2>\n

    Os TNE funcionantes do p\u00e2ncreas s\u00e3o: insulinoma, gastrinoma, glucagonoma, vipoma e somatostatinoma.<\/p>\n

    Maioria dos\u00a0TNE-P s\u00e3o malignos, exce\u00e7\u00e3o aos insulinomas e TNE-NF menores que 2 cm.<\/p>\n

    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

    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

    Tumores pancre\u00e1ticos m\u00faltiplos s\u00e3o raros e devem despertar a suspeita de NEM1.<\/p>\n

    A SEGUIR VAMOS VER AS PRINCIPAIS CARACTER\u00cdSTICAS DE CADA SUBTIPO HISTOL\u00d3GICO<\/strong><\/p>\n

    INSULINOMAS<\/strong><\/h3>\n
      \n
    • \u00c9 o TNE mais frequente das ilhotas pancre\u00e1ticas.<\/li>\n
    • 90% s\u00e3o benignos, por\u00e9m s\u00e3o sintom\u00e1ticos mesmo quando pequenos.<\/li>\n
    • Cerca de 10% est\u00e3o associadas a NEM.<\/li>\n
    • S\u00e3o les\u00f5es hipervascularizadas e solit\u00e1rias, frequentemente < 2 cm.<\/li>\n
    • Tr\u00edade de Whipple:<\/strong>\n
        \n
      • hipoglicemia (< 50)<\/li>\n
      • sintomas neuroglicopenicos (borramento visual, fraqueza, cansa\u00e7o, cefaleia, sonol\u00eancia)<\/li>\n
      • desaparecimento dos sintomas com a reposi\u00e7\u00e3o de glicose<\/li>\n<\/ul>\n<\/li>\n
      • insulina s\u00e9rica > 6 UI\/ml<\/li>\n
      • Pept\u00eddeo C > 0,2 mmol\/l<\/li>\n
      • Pr\u00f3-insulina > 5 UI\/ml<\/li>\n
      • Teste de jejum prolongado positivo (99% dos casos)<\/li>\n<\/ul>\n

        \u00a0<\/strong><\/p>\n

        GASTRINOMAS<\/strong><\/h3>\n
          \n
        • \u00c9 mais comum no duodeno, mas 30% dos casos est\u00e3o no p\u00e2ncreas<\/li>\n
        • S\u00e3o os TNE do p\u00e2ncreas mais frequentes depois dos insulinomas.<\/li>\n
        • Est\u00e3o associados a Sd. NEM 1 em 30%, e nesses casos se apresentam como les\u00f5es pequenas e multifocais.<\/li>\n
        • Provocam hipergastrinemia e s\u00edndrome de Zollinger-Ellison.<\/li>\n
        • 60% s\u00e3o malignos.<\/li>\n
        • Tratamento: cir\u00fargico nos espor\u00e1dicos (DPT).<\/li>\n
        • 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

          <\/h3>\n

          GLUCAGONOMAS<\/strong><\/h3>\n
            \n
          • Raros;\u00a0\u00a0maioria\u00a0espor\u00e1dicos.<\/li>\n
          • Geralmente, s\u00e3o grandes e solit\u00e1rios, com tamanho entre 3-7 cm ocorrendo principalmente na cauda do p\u00e2ncreas.<\/li>\n
          • Sintomas: eritema\u00a0necrol\u00edtico\u00a0migrat\u00f3rio (80%), DM, desnutri\u00e7\u00e3o, perda de peso, tromboflebite, glossite, queilite angular, anemia<\/li>\n
          • Crescimento lento e sobrevida longa<\/li>\n
          • Met\u00e1stase linfonodal ou hep\u00e1tica ocorre em 60-75% dos casos.<\/li>\n<\/ul>\n

            VIPOMAS<\/strong><\/h3>\n
              \n
            • Extremamente raros<\/li>\n
            • Como os glucagonomas, localizados na cauda, grandes e solit\u00e1rios.<\/li>\n
            • Maioria maligno e metast\u00e1tico<\/li>\n
            • Em 10% dos casos pode ser extra-pancre\u00e1tico.<\/li>\n
            • Quadro cl\u00ednico\u00a0relacionada a secre\u00e7\u00e3o do VIP (pept\u00eddeo vasoativo intestinal):\n
                \n
              • diarr\u00e9ia\u00a0(mais de 3L litros por dia) \u2013 \u00e1gua de lavado de arroz<\/li>\n
              • Dist\u00farbios hidro-eletrol\u00edtico: hipocalemia, hipocloridria, acidose metab\u00f3lica<\/li>\n
              • Rubor<\/li>\n<\/ul>\n<\/li>\n
              • Excelente resposta ao tratamento com an\u00e1logos da somatostatina.<\/li>\n<\/ul>\n

                \u00a0<\/strong><\/p>\n

                SOMATOSTATINOMAS<\/strong><\/h3>\n
                  \n
                • \u00c9 o menos comum de todos<\/li>\n
                • Somatostatina leva\u00a0a\u00a0inibi\u00e7\u00e3o da secre\u00e7\u00e3o end\u00f3crina e ex\u00f3crina e afeta a motilidade intestinal.<\/li>\n
                • Les\u00e3o\u00a0solit\u00e1ria, grande, espor\u00e1dico, maioria maligno e metastatico<\/li>\n
                • Quadro cl\u00ednico:\n
                    \n
                  • Diabetes (75%)<\/li>\n
                  • Colelit\u00edase (60%)<\/li>\n
                  • Esteatorr\u00e9ia (60%)<\/li>\n
                  • Perda de peso<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n

                    TNE N\u00c3O FUNCIONANTES DO P\u00c2NCREAS<\/strong><\/h3>\n
                      \n
                    • 20% de todos os TNEs do p\u00e2ncreas.<\/li>\n
                    • 50% s\u00e3o malignos.<\/li>\n
                    • O principal diagn\u00f3stico diferencial \u00e9 com o adenocarcinoma<\/strong><\/li>\n<\/ul>\n

                      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 menores que 1 cm<\/strong> e conduta individualizada<\/u>, entre 1-2 cm.<\/p>\n

                      TNE PANCRE\u00c1TICO RELACIONADOS A SINDROMES HEREDITARIAS<\/strong><\/h3>\n
                        \n
                      • 10% dos TNE-P s\u00e3o relacionados a NEM-1<\/li>\n
                      • Frequentemente multic\u00eantricos<\/strong>,<\/li>\n
                      • Geralmente acometendo pessoas mais jovens.<\/li>\n
                      • Geralmente de comportamento benigno, por\u00e9m apresentam potencial maligno<\/li>\n
                      • Gastrinoma 30-40%; Insulinoma 10%; TNE-NF 20-50%; outros 2%<\/li>\n
                      • Tto cir\u00fargico \u00e9 controverso, pq as vezes n\u00e3o controla a gastrinemia (tumores m\u00faltiplos)<\/li>\n<\/ul>\n

                        PS: Voc\u00ea se recorda das neoplasias neuroend\u00f3crinas m\u00faltiplas?\u00a0<\/em><\/h4>\n

                        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

                        NEM-1<\/h5>\n
                          \n
                        • Doen\u00e7a autoss\u00f4mica dominante<\/li>\n
                        • Predispoe a TU (3Ps): Paratire\u00f3ide; Pituit\u00e1ria (hip\u00f3fise); P\u00e2ncreas,<\/li>\n
                        • Geralmente de comportamento benigno, por\u00e9m apresentam potencial maligno<\/li>\n
                        • Gastrinoma 30-40%; Insulinoma 10%; TNE-NF 20-50%; outros 2%<\/li>\n
                        • Tto cir\u00fargico \u00e9 controverso, pq as vezes n\u00e3o controla a gastrinemia (tumores m\u00faltiplos)<\/li>\n<\/ul>\n
                          NEM-2A:<\/h5>\n
                            \n
                          • Carcinoma medular da tireoide,<\/li>\n
                          • Feocromocitoma,<\/li>\n
                          • Hiperplasia ou adenomas das gl\u00e2ndulas paratireoides (com consequente hiperparatireoidismo).<\/li>\n<\/ul>\n
                            NEM-2B:<\/h5>\n
                              \n
                            • Carcinoma medular de tireoide,<\/li>\n
                            • Feocromocitoma<\/li>\n
                            • Neuromas mucosos e intestinais m\u00faltiplos<\/li>\n<\/ul>\n

                              Refer\u00eancias:<\/strong><\/h3>\n
                                \n
                              1. Pathology, classification, and grading of neuroendocrine neoplasms arising in the digestive system – UpToDate ; 2021<\/span><\/li>\n
                              2. 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

                                Como citar esse artigo:<\/h3>\n

                                Martins BC, de Moura DTH. Tumores neuroend\u00f3crinos do pancreas. Endoscopia Terap\u00eautica. 2022; vol 1.\u00a0 Dispon\u00edvel em: endoscopiaterapeutica.net\/pt\/assuntosgerais\/tumores-neuroendocrinos-do-pancreas<\/p>\n","protected":false},"excerpt":{"rendered":"

                                Introdu\u00e7\u00e3o A incid\u00eancia dos tumores neuroend\u00f3crinos do p\u00e2ncreas est\u00e1 crescendo, possivelmente devido \u00e0 realiza\u00e7\u00e3o…<\/p>\n","protected":false},"author":712,"featured_media":10912,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"","_lmt_disable":"","footnotes":""},"categories":[151],"tags":[311,313,314,315,310,312],"ano":[309],"tipo":[155,154,159],"volume":[263],"class_list":["post-10908","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-assuntosgerais","tag-glucagonoma","tag-insulinoma","tag-nem","tag-nem-1","tag-tumor-neuroendocrino","tag-vipoma","ano-309","tipo-cpre","tipo-ecoendoscopia","tipo-outros","volume-volume-i"],"yoast_head":"\nTumores neuroend\u00f3crinos do pancreas • Endoscopia Terapeutica<\/title>\n<meta name=\"description\" content=\"Esse artigo do Endoscopia Terap\u00eautica 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