Head and Neck Paragangliomas: Management of 32 cases including Peptide Receptor Radionuclide Therapy

EnglishBackground: Paragangliomas (PG) account for 0.6% of all tumors in the head and neck region. Widely distributed, predilection for carotid body, jugular foramen, tympanic plexus and vagus nerve ganglia is observed. The management of these tumors remains a challenge with three possible options: surgery, radiation therapy and wait-and-see policy. Regardless treatment must be individualized. Aim: We conducted a revision of all paragangliomas treated in the Otolaryngology Head and Neck Surgery Department of the Portuguese Institute of Oncology, in Porto. The aim of this analysis is to characterize clinical features, investigations until diagnosis, type of treatment and results. Methods: Retrospective study (Analysis of 40 years). Results: Our population involved 32 patients aged between 25 to 75 years who had been treated in the last 40 years. Of the 32 PG, 24 were jugulotympanic; 6 were carotid body lesions and 2 vagal paragangliomas. One patient has bilateral and multiple tumors. Just 2 cases were hereditary. Conclusion: Clinical presentation and histological diversity makes the management of head and neck paragangliomas a challenge. Although in cases with similar histology, there is no consensus between surgery and radiotherapy as treatment options. Therefore we consider that treatment must be individualized, taking into account patient`s age, tumor localization and size, multicentricity, and preexisting cranial nerve deficits. PRRT (Peptide Receptor Radionuclide Therapy), an investigational option adopted for some of our cases, could be an alternative or instead a complementary treatment modality for paragangliomas in several stages. portuguesIntroducao: Os tumores glomicos, tambem denominados por paragangliomas (PG) correspondem a 0,6% dos tumores da regiao da cabeca e pescoco. Nesta area geralmente localizam-se na bifurcacao carotidea , no gânglio nodoso do nervo vago ou a nivel do osso temporal. A vigilância clinica, a radioterapia e a exerese cirurgica com ou sem embolizacao previa, sao consideradas opcoes terapeuticas validas, pelo que, cada caso devera ser individualizado. Objectivo: Analisar os casos estudados e tratados no Instituto Portugues de Oncologia do Porto (IPOPorto). Esta revisao visa descrever as manifestacoes clinicas, a etiopatogenia, o diagnostico, o tratamento e o follow-up. Material e Metodos: Estudo retrospectivo (40 anos de experiencia) Resultado: A nossa amostra e constituida por 32 doentes com diagnostico de paragangliomas da regiao da cabeca e pescoco, com idades compreendidas entre 25 a 75 anos, que foram submetidos a tratamento no IPO-Porto, tendo em consideracao os ultimos 40 anos. Dos 32 paragangliomas: 24 sao jugulo-timpânicos; 6 localizam-se no corpo carotideo; e 2 sao paragangliomas vagais. Um doente apresentou tumores multiplos e bilaterais. Em dois casos foi detectada anomalia genetica. Conclusao: O tratamento dos PG continua a ser foco de discussao na literatura medica. A cirurgia e geralmente o tratamento de eleicao, no entanto, em casos particulares outras modalidades de tratamento, primario ou complementar, poderao ser implementadas. A terapeutica com radiofarmacos e uma opcao promissora no tratamento destes tumores.