头颈副神经节瘤 (HNPGL) 是一种罕见的肿瘤，其中大约一半是由琥珀酸脱氢酶基因 (SDHx) 的种系致病性变异 (PV) 引起的。 HNPGL 患者具有异质性的复发和转移倾向。因此，我们的目的是评估患有或不患有 SDHx 相关 HNPGL 的患者复发 (RD) 和/或转移性疾病的患病率和预测因素。这项横断面研究使用了 6 个转诊中心登记的 214 名患者的回顾性数据。数据包括性别、年龄、原发肿瘤治疗、位置和大小、生化表型、种系 PV、RD（局部或新肿瘤）的存在和/或转移。有和没有 SDHx 相关 HNPGL 的患者分别为 74% 和 40% RD 的患病率分别为没有 SDHx 相关 HNPGL 的患者仅在头颈部区域出现复发性肿瘤。整个队列中 RD 的唯一独立预测因子是 SDHx PV 的存在。转移率达到9%-13%。对于患有 SDHx 相关 HNPGL 的患者，肿瘤尺寸较大（>2.3 cm，OR：50.0，CI：2.6-977.6），初次诊断时年龄较小（<42 岁，OR：27.3，CI：1.8-407.2），并且存在SDHB PV（OR：15.6；CI：1.5-164.8）是转移的独立预测因子。对于没有 SDHx 相关 HNPGL 的患者，只有颈动脉体位置是转移的独立预测因子（OR：18.9，CI：2.0-182.5）。没有 SDHx 相关 HNPGL 的患者由于 RD 患病率较高，需要长期随访成像主要局限于头颈区域。由于颈动脉体 HNPGL 在散发性肿瘤中具有最高的转移风险，因此建议在获得基于人群的数据之前进行频繁随访的根治性治疗。重要的是，患有 SDHx 相关 HNPGL 的患者可能会在肿瘤较小时受益于早期根治性治疗，以降低转移风险。© 作者 2024。由牛津大学出版社代表欧洲内分泌学会出版。版权所有。如需商业重复使用，请联系 reprints@oup.com 获取转载和转载的翻译权。所有其他权限都可以通过我们网站文章页面上的权限链接通过我们的 RightsLink 服务获得 - 如需了解更多信息，请联系journals.permissions@oup.com。

Head-neck paragangliomas (HNPGLs) are rare tumors with approximately half arising due to germline pathogenic variants (PVs) in succinate dehydrogenase genes (SDHx). Patients with HNPGL have heterogeneous propensity to recur and metastasize. Thus, we aim to assess prevalence and predictors of recurrent (RD) and/or metastatic disease in patients with and without SDHx-related HNPGLs.This cross-sectional study used retrospective data of 214 patients enrolled in six referral centers. Data included sex, age, primary tumor treatment, location, and size, biochemical phenotype, germline PVs, presence of RD (locoregional or new tumor), and/or metastasis.Patients with and without SDHx-related HNPGLs showed 74% and 40% prevalence of RD, respectively. Patients without SDHx-related HNPGLs presented with recurrent tumors only in head-neck regions. The only independent predictor for RD in the entire cohort was presence of SDHx PVs. Metastatic prevalence reached 9%-13%. For patients with SDHx-related HNPGLs, large tumor size (>2.3 cm, OR:50.0, CI:2.6-977.6), young age at initial diagnosis (<42years, OR:27.3, CI:1.8-407.2), and presence of SDHB PV (OR:15.6; CI:1.5-164.8) were independent predictors of metastasis. For patients without SDHx-related HNPGLs, only carotid-body location was an independent predictor of metastasis (OR:18.9, CI:2.0-182.5).Patients without SDHx-related HNPGLs require long-term follow-up due to high prevalence of RD with imaging largely restricted to head-neck regions. As carotid-body HNPGLs have the highest metastatic risk among sporadic tumors, radical treatment with frequent follow-up is suggested until population-based data are available. Importantly, patients with SDHx-related HNPGLs might benefit from early radical treatment when tumors are still small to reduce metastatic risk.© The Author(s) 2024. Published by Oxford University Press on behalf of European Society of Endocrinology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.