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

Authors

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

Keywords:

Carotid Body Tumor, Cranial Nerve Neoplasms, Extra-Adrenal Paraganglioma, Head and Neck Neoplasms, Paraganglioma

Abstract

Introduction: Paragangliomas are usually benign slow-growing tumors, but they are locally invasive and can cause significant morbidity. The aim of this study was to characterize the presenting symptoms, secretory status, genetics, imaging features, treatment modalities, post-treatment complications and survival of patients with head and neck paragangliomas treated at a single institution.
Material and Methods: We retrospectively reviewed the clinical records of patients managed at our center between 1997 and 2020.
Results: Seventy-three patients were included in the study, encompassing 89 head and neck paragangliomas. Forty-eight patients (65.8%) were female and 15 (20.5%) had multiple tumor sites (including 10 patients with multicentric benign paragangliomas and five with disseminated malignant disease). Regarding location, our series encompassed 40 temporal bone paragangliomas (44.9%), 24 carotid body paragangliomas (27%), 22 vagal paragangliomas (24.7%), two laryngeal paragangliomas (2.2%) and one sinonasal paraganglioma (1.1%). Excessive catecholamine secretion was detected in 11 patients (15.1%). Sixty-four patients (87.7%) underwent genetic testing. Of those, 24 (37.5%) exhibited pathogenic succinate dehydrogenase complex germline mutations. Regarding patients who presented with untreated disease, 45 patients (66.2%), encompassing 55 tumors, underwent surgery as primary treatment modality, 20 (29.4%; 23 tumors) were initially treated with radiotherapy and three patients (4.4%, encompassing three solitary tumors) were kept solely under watchful waiting. Five-year overall survival was 94.9% and disease-free survival was 31.9%.
Conclusion: Head and neck paragangliomas are rare, slow-growing but locally aggressive tumors resulting in high morbidity but low mortality rates.

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