Head and neck paragangliomas: the experience of a southern European cancer center

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

doi:10.20344/amp.17185

Head and neck paragangliomas: the experience of a southern European cancer center

Título traduzido da contribuição: 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

^*Autor correspondente para este trabalho

Resultado de pesquisa › revisão de pares

2 Citações (Scopus)

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 clíni-cas, 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 la-ríngeos (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.

Título traduzido da contribuição	Paragangliomas da cabeça e pescoço: a experiência de um centro oncológico do sul da Europa
Idioma original	English
Páginas (de-até)	789-797
Número de páginas	9
Revista	Acta Medica Portuguesa
Volume	35
Número de emissão	13
DOIs	https://doi.org/10.20344/amp.17185
Estado da publicação	Publicado - 5 mar. 2022
Publicado externamente	Sim

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Este resultado contribui para o(s) seguinte(s) Objetivo(s) de Desenvolvimento Sustentável

ODS 3 Boa saúde e bem-estar

Keywords

Neoplasias de cabeça e pescoço
Neoplasias dos nervos cranianos
Paraganglioma
Paraganglioma extrassu-prarrenal
Tumor do corpo carotídeo

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10.20344/amp.17185Licença:

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Citação

@article{bf796888129e4f459eb9708a79e76385,

title = "Head and neck paragangliomas: the experience of a southern European cancer center",

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.",

keywords = "Carotid body tumor, Cranial nerve neoplasms, Extra-adrenal paraganglioma, Head and neck neoplasms, Paraganglioma, Neoplasias de cabe{\c c}a e pesco{\c c}o, Neoplasias dos nervos cranianos, Paraganglioma, Paraganglioma extrassu-prarrenal, Tumor do corpo carot{\'i}deo",

author = "Lu{\'i}s Castelhano and Filipe Correia and Sara Donato and L{\'i}gia Ferreira and Pedro Montalv{\~a}o and Miguel Magalh{\~a}es",

note = "Publisher Copyright: Copyright {\textcopyright} Ordem dos M{\'e}dicos 2022",

year = "2022",

month = mar,

day = "5",

doi = "10.20344/amp.17185",

language = "English",

volume = "35",

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journal = "Acta Medica Portuguesa",

issn = "0870-399X",

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}

TY - JOUR

T1 - Head and neck paragangliomas

T2 - the experience of a southern European cancer center

AU - Castelhano, Luís

AU - Correia, Filipe

AU - Donato, Sara

AU - Ferreira, Lígia

AU - Montalvão, Pedro

AU - Magalhães, Miguel

PY - 2022/3/5

Y1 - 2022/3/5

N2 - 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.

AB - 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.

KW - Carotid body tumor

KW - Cranial nerve neoplasms

KW - Extra-adrenal paraganglioma

KW - Head and neck neoplasms

KW - Paraganglioma

KW - Neoplasias de cabeça e pescoço

KW - Neoplasias dos nervos cranianos

KW - Paraganglioma

KW - Paraganglioma extrassu-prarrenal

KW - Tumor do corpo carotídeo

UR - https://www.scopus.com/pages/publications/85127612536

U2 - 10.20344/amp.17185

DO - 10.20344/amp.17185

M3 - Article

C2 - 35290759

AN - SCOPUS:85127612536

SN - 0870-399X

VL - 35

SP - 789

EP - 797

JO - Acta Medica Portuguesa

JF - Acta Medica Portuguesa

IS - 13

ER -

Head and neck paragangliomas: the experience of a southern European cancer center

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