Carcinoma Medular da Tiroide – perspetiva após as guidelines de 2015 da ATA

  • Ana Margarida Martins Faculdade de Medicina da Universidade do Porto
  • João Capela Faculdade de Medicina da Universidade do Porto; Serviço de Cirurgia Geral- Centro Hospitalar de S. João

Resumo

O Carcinoma medular da tiroide (CMT) é um tumor neuroendócrino raro (1-2% de todos os tumores da tiroide), que surge a partir de células C, produtoras de calcitonina. A sua grande maioria (75%) corresponde a formas esporádicas, no entanto, existem também formas hereditárias (25%) que fazem parte da síndrome de neoplasia endócrina múltipla do tipo 2, tipo 2A e 2B. Todos os doentes com estas síndromes apresentam uma mutação germinativa do proto-oncogene RET e cerca de 50% dos doentes com CMT esporádico, mutações somáticas deste mesmo gene. O diagnóstico e tratamento precoces são essenciais. O gold standard para diagnóstico de CMT é a biópsia aspirativa com agulha fina (BAAF) de um nódulo tiroideu suspeito, juntamente com o doseamento sérico de calcitonina. O teste genético, com pesquisa de mutações do gene RET, deve ser realizado a todos os doentes. O único tratamento curativo do CMT é a tiroidectomia total com esvaziamento dos compartimentos ganglionares cervicais necessários. O tratamento mais indicado em doentes com doença residual, recorrente ou metastática ainda está por esclarecer, uma vez que a maioria dos doentes tem uma baixa progressão da doença durante vários anos. Assim, prognóstico é na maior parte das vezes relativamente bom, sendo que a principal causa de morte é a metastização à distância. Recentemente, foram aprovados dois inibidores da tirosinacinase, o vandetanib e o cabozantinib, para o tratamento de CMT agressivo e sintomático e existem já vários outros inibidores em ensaios clínicos. Estes agentes são inibidores estáticos da doença, não tendo ação lítica ou destrutiva. Por essa razão, são necessários mais estudos no sentido de compreender as vias celulares implicadas na etiopatogenia desta doença e consequentemente encontrar nova terapêuticas sistémicas dirigidas no sentido de tratar definitivamente a doença metastática. O objetivo deste artigo de revisão é abordar a etiopatogenia, a apresentação clínica, o diagnóstico, o estadiamento, os testes genéticos e fundamentalmente o tratamento do CMT, tendo em conta as evidências mais recentes.

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Biografias Autor

Ana Margarida Martins, Faculdade de Medicina da Universidade do Porto

Aluna de Mestrado Integrado de Medicina – Departamento de Cirurgia e Fisiologia – Faculdade de Medicina da Universidade do Porto

 

João Capela, Faculdade de Medicina da Universidade do Porto; Serviço de Cirurgia Geral- Centro Hospitalar de S. João

Assistente Hospitalar Graduado da Unidade de Cirurgia Endócrina e Cervical do Serviço de Cirurgia Geral do Hospital São João, Porto, Portugal

Mestre em Medicina Molecular pela Faculdade de Medicina do Porto

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Publicado
2017-12-31
Como Citar
MARTINS, Ana Margarida; CAPELA, João. Carcinoma Medular da Tiroide – perspetiva após as guidelines de 2015 da ATA. Revista Portuguesa de Cirurgia, [S.l.], n. 43, dez. 2017. ISSN 2183-1165. Disponível em: <https://revista.spcir.com/index.php/spcir/article/view/633>. Acesso em: 21 nov. 2018.
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