Author(s)
Luís Guerra Castelhano, MD
Filipe Correia
Marta Mariano
Lígia Ferreira, MD
Pedro Montalvão, MD
Pedro Montalvão, MD
Miguel Magalhães, MD
Affiliation(s)
Hospital de Egas Moniz; Instituto Português de Oncologia Lisboa; Department of Otorhinolaryngology - Head and Neck surgery, IPO Lisboa;
Abstract:
Objectives: To characterize the presenting symptoms, secretory status, genetical and imagiological workups, treatment modalities, post-treatment complications and survival of patients with head and neck paragangliomas treated at a single institution.
Material and methods: Retrospective review of the clinical records of patients managed at our center between 1997 and 2020.
Results: Seventy-three patients were included in the study, comprehending 89 tumors. Forty-eight patients (65.8%) were female and 15 (20.5%) had multiple tumor sites (including 10 patients with multicentric benign head and neck paragangliomas and 5 with disseminated malignant disease). Regarding location, our series comprehended 40 temporal bone paragangliomas (44.9%), 24 carotid body paragangliomas (27%), 22 vagal paragangliomas (24.7%), 2 laryngeal paraganliomas (2.2%) and 1 sinonasal paraganglioma (1.1%). Excessive catecholamine secretion was detected in 11 patients (15.1%). Twenty-four patients (32.8%) exhibited likely pathogenic and pathogenic succinate dehydrogenase complex germline mutations. Regarding patients who presented with untreated disease, 45 patients (66.2%), comprehending 55 tumors, underwent surgery as primary modality of treatment, 20 (29.4%; 23 tumors) were initially treated with radiotherapy and 3 (4.4%, comprehending 3 solitary tumors) were kept solely under watchful waiting. Five-year overall survival was 94.9% and disease-free survival was 31.9%.
Conclusions: Current practice demands a battery of complementary tests before advising a therapeutic option. Genetic testing is now widely recommended in order to diagnose a potentially morbid pathology at a presymptomatic stage. A lifelong commitment is mandatory as rehabilitation of surgical sequelae and surveillance play a fundamental role in appropriate care.