Paragangliomas da Cabeça e Pescoço: A Experiência de um Centro Oncológico do Sul da Europa

Luís Castelhano; Filipe Correia; Sara Donato; Lígia Ferreira; Pedro Montalvão; Miguel Magalhães

doi:10.20344/amp.17185

Autores

Luís Castelhano Serviço de Otorrinolaringologia. Hospital de Egas Moniz. Centro Hospitalar Lisboa Ocidental. Lisboa. https://orcid.org/0000-0001-6501-6941
Filipe Correia Serviço de Otorrinolaringologia. Hospital de Egas Moniz. Centro Hospitalar Lisboa Ocidental. Lisboa. https://orcid.org/0000-0002-5092-5621
Sara Donato Serviço de Endocrinologia. Instituto Português de Oncologia Francisco Gentil. Lisboa. https://orcid.org/0000-0001-8704-5292
Lígia Ferreira Serviço de Otorrinolaringologia. Instituto Português de Oncologia Francisco Gentil. Lisboa. https://orcid.org/0000-0001-7268-7495
Pedro Montalvão Serviço de Otorrinolaringologia. Instituto Português de Oncologia Francisco Gentil. Lisboa.
Miguel Magalhães Serviço de Otorrinolaringologia. Instituto Português de Oncologia Francisco Gentil. Lisboa.

DOI:

https://doi.org/10.20344/amp.17185

Palavras-chave:

Neoplasias de Cabeça e Pescoço, Neoplasias dos Nervos Cranianos, Paraganglioma, Paraganglioma Extrassuprarrenal, Tumor do Corpo Carotídeo

Resumo

Introdução: Os paragangliomas apresentam frequentemente um comportamento benigno e um crescimento indolente. Apesar disso, são localmente invasivos, podendo causar morbilidade significativa. O objetivo deste trabalho foi descrever as manifestações clínicas, atividade secretora, estudos genéticos e imagiológicos, modalidades terapêuticas, complicações e sobrevivência dos doentes com paragangliomas da cabeça e pescoço.
Material e Métodos: Estudo retrospetivo dos doentes com paragangliomas da cabeça e pescoço observados num centro hospitalar terciário entre 1997 e 2020.
Resultados: Foram incluídos no estudo 73 doentes, englobando 89 paragangliomas. Quarenta e oito doentes (65,8%) eram do sexo feminino e 15 (20,5%) apresentavam múltiplos focos tumorais (10 por multicentricidade e cinco por doença maligna disseminada). Foram incluídos 40 paragangliomas do osso temporal (44,9%), 24 tumores do corpo carotídeo (27%), 22 vagais (24,7%), dois laríngeos (2,2%) e um nasossinusal (1,1%). A secreção excessiva de catecolaminas foi detetada em 11 doentes (15,1%). Sessenta e quatro doentes (87,7%) foram alvo de teste genético. Desses, 24 (37,5%) exibiram mutações patogénicas do complexo succinato desidrogenase. Dos doentes com doença primária, 45 (66,2%; 55 tumores) foram submetidos a tratamento cirúrgico, 20 (29,4%; 23 tumores) a radioterapia e três (4,4%) ficaram sob vigilância. Aos cinco anos, a sobrevida global foi de 94,9% e a sobrevida livre de doença foi de 31,9%.
Conclusão: Os paragangliomas da cabeça e pescoço são tumores raros, de crescimento lento, mas localmente agressivos que resultam em elevadas taxas de morbilidade, mas baixas taxas de mortalidade.

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