Serviço de Otorrinolaringologia. Hospital de Egas Moniz. Centro Hospitalar Lisboa Ocidental. Lisboa. Portugal.
Serviço de Endocrinologia. Instituto Português de Oncologia Francisco Gentil. Lisboa. Portugal.
Acta Med Port. 2022 Nov 2;35(11):789-797. doi: 10.20344/amp.17185. Epub 2022 Mar 15.
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.
We retrospectively reviewed the clinical records of patients managed at our center between 1997 and 2020.
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%.
Head and neck paragangliomas are rare, slow-growing but locally aggressive tumors resulting in high morbidity but low mortality rates.
副神经节瘤通常为良性、生长缓慢的肿瘤，但具有局部侵袭性，可导致严重的发病率。本研究的目的是描述在单一机构接受治疗的头颈部副神经节瘤患者的临床表现、分泌状态、遗传学、影像学特征、治疗方式、治疗后并发症和生存情况。
我们回顾性分析了我们中心在 1997 年至 2020 年期间治疗的患者的临床记录。
本研究共纳入 73 例患者，共 89 例头颈部副神经节瘤。48 例（65.8%）为女性，15 例（20.5%）有多发性肿瘤（包括 10 例多发性良性副神经节瘤和 5 例转移性恶性疾病）。就位置而言，我们的研究包括 40 例颞骨副神经节瘤（44.9%）、24 例颈动脉体副神经节瘤（27%）、22 例迷走神经副神经节瘤（24.7%）、2 例喉部副神经节瘤（2.2%）和 1 例鼻旁窦副神经节瘤（1.1%）。11 例（15.1%）患者存在儿茶酚胺分泌过多。64 例患者（87.7%）接受了基因检测。其中，24 例（37.5%）存在致病性琥珀酸脱氢酶复合物种系突变。对于初诊时未治疗的患者，45 例（66.2%），共 55 个肿瘤，采用手术作为主要治疗方式，20 例（29.4%；23 个肿瘤）采用放疗作为初始治疗方式，3 例（4.4%；3 个孤立肿瘤）仅接受密切观察。5 年总生存率为 94.9%，无疾病生存率为 31.9%。
头颈部副神经节瘤是罕见的、生长缓慢但具有局部侵袭性的肿瘤，导致高发病率，但死亡率低。
