Lamas Cristina, Febrero Beatriz, Casteràs Anna, Romero-Lluch Ana, Recio-Córdova José María, Ros Inmaculada, Iglesias Pedro, Hanzu Felicia A, Araujo-Castro Marta, Guerrero-Pérez Fernando, Ares Jessica, Menéndez Edelmiro, Paja Miguel, Álvarez-Escolá Cristina, Librizzi Soledad, Sánchez-Sobrino Paula, Andres-Pretel Fernando, Calatayud María
Endocrinology Department, Complejo Hospitalario Universitario de Albacete, Albacete, Spain.
Facultad de Medicina, Universidad de Castilla-La Mancha, Albacete, Spain.
Otolaryngol Head Neck Surg. 2025 Jan;172(1):176-183. doi: 10.1002/ohn.1012. Epub 2024 Oct 13.
OBJECTIVE, STUDY DESIGN, SETTING, METHODS: To improve our knowledge about head and neck paragangliomas (HN-PGL), the clinical characteristics of all the patients with HN-PGL included in the Spanish Registry were analyzed, as well as the treatment modalities and their outcomes.
A total of 202 patients, 67.8% women, aged 53.1 ± 17.4, with 264 HN-PGL, from 16 Spanish hospitals, were included. Tumors were located in the carotid body in 61%, jugular bulb in 20.5%, tympanic area in 10.2%, and along the vagal nerve in 6.4%. Multiple tumors developed in 20.8% and metastatic disease in 4%. A genetic study was done in 64.4% and showed a pathogenic variant in SDHx in 50%. These patients were younger, with no sex predominance, and had more multiple and metastatic tumors. Tumors were treated by surgery in 134 patients, radiotherapy in 33, and other treatments in 20, and were observed without active treatment in 41 patients, who were older, more often asymptomatic, and had smaller tumors. Tumors treated with radiotherapy were larger and more often in locations other than the carotid body. After a median follow-up of 80 months (interquartile range: 41-136), 6.9% had died; among the survivors, 48.4% were disease-free, 42.1% stable, and 9.4% had progressed. The rate of sequelae was similar among patients submitted to surgery, radiotherapy, or observation. We could not identify any prognostic factor for progression.
Most HN-PGL are localized slow-growing tumors. Long-term survival is high, even in case of metastasis. Although surgery is the most common treatment, radiotherapy and active surveillance are safe approaches.
目的、研究设计、研究地点、方法：为增进我们对头颈部副神经节瘤（HN-PGL）的了解，分析了西班牙登记处纳入的所有HN-PGL患者的临床特征、治疗方式及其结果。
共纳入来自16家西班牙医院的202例患者，女性占67.8%，年龄53.1±17.4岁，共有264个HN-PGL。肿瘤位于颈动脉体的占61%，颈静脉球的占20.5%，鼓室区的占10.2%，沿迷走神经的占6.4%。20.8%的患者发生多发肿瘤，4%发生转移。64.4%的患者进行了基因研究，其中50%显示SDHx存在致病变异。这些患者较年轻，无性别优势，多发和转移肿瘤更多。134例患者接受手术治疗，33例接受放射治疗，20例接受其他治疗，41例患者未进行积极治疗而是接受观察，这些患者年龄较大，更常无症状，肿瘤较小。接受放射治疗的肿瘤较大，且更多位于颈动脉体以外的部位。中位随访80个月（四分位间距：41 - 136个月）后，6.9%的患者死亡；在幸存者中，48.4%无疾病，42.1%病情稳定，9.4%病情进展。接受手术、放疗或观察的患者后遗症发生率相似。我们未能确定任何进展的预后因素。
大多数HN-PGL是局限性生长缓慢的肿瘤。即使发生转移，长期生存率也很高。虽然手术是最常见的治疗方法，但放疗和主动监测也是安全的方法。
