Clinical management and outcome of head and neck paragangliomas (HNPGLs): A single centre retrospective study

IF 3 3区医学 Q2 ENDOCRINOLOGY & METABOLISM

Clinical Endocrinology Pub Date : 2024-05-02 DOI:10.1111/cen.15070

Milad Darrat, Louis Lau, Colin Leonard, Stephen Cooke, Muhammad A. Shahzad, Claire McHenry, David R. McCance, Steven J. Hunter, Karen Mullan, John R. Lindsay, Una Graham, Neil Bailie, Susie Hampton, Simon Rajendran, Fionnuala Houghton, David Conkey, Patrick J. Morrison, Philip C. Johnston

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引用次数: 0

Abstract

Context

Head and neck paragangliomas (HNPGLs) are rare, usually benign, slow-growing tumours arising from neural crest-derived tissue. Definitive management pathways for HNPGLs have yet to be clearly defined.

Objective

To review our experience of the clinical features and management of these tumours and to analyse outcomes of different treatment modalities.

Methods

Demographic and clinical data were obtained from The Northern Ireland Electronic Care Record (NIECR) as well from a prospectively maintained HNPGL database between January 2011 through December 2023.

Results

There were 87 patients; 50 females: 37 males with a mean age of 52.3 ± 14.2 years old (range 17–91 years old). 58.6% (n = 51) of patients had carotid body tumours, 25.2% (n = 22) glomus vagal tumours, 6.8% (n = 6) tumours in the middle ear, 2.2% (n = 2) in the parapharyngeal space and 1.1% (n = 1) in the sphenoid sinus. 5.7% (n = 5) of patients had multifocal disease. The mean tumour size at presentation was 3.2 ± 1.4 cm (range 0.5–6.9 cm). Pathogenic SDHD mutations were identified in 41.3% (n = 36), SDHB in 12.6% (n = 11), SDHC in 2.2% (n = 2) and SDHA in 1.1% (n = 1) of the patients. Overall treatment modalities included surgery alone in 51.7% (n = 45) of patients, radiotherapy in 14.9% (n = 13), observation in 28.7% (n = 25), and somatostatin analogue therapy with octreotide in 4.5% (n = 4) of patients. Factors associated with a significantly higher risk of recurrence included age over 60 years (p = .04), tumour size exceeding 2 cm (p = .03), positive SDHx variants (p = .01), and vagal and jugular tumours (p = .04).

Conclusion

The majority of our patients underwent initial surgical intervention and achieved disease stability. Our results suggest that carefully selected asymptomatic or medically unfit patients can be safely observed provided lifelong surveillance is maintained. We advocate for the establishment of a UK and Ireland national HNPGL registry, to delineate optimal management strategies for these rare tumours and improve long term outcomes.

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头颈部副神经节瘤（HNPGLs）的临床管理和疗效：单中心回顾性研究

背景头颈部副神经节瘤（HNPGL）是一种罕见的肿瘤，通常为良性，生长缓慢，来源于神经嵴组织。方法从北爱尔兰电子病历（NIECR）以及前瞻性维护的HNPGL数据库中获取人口统计学和临床数据，时间跨度为2011年1月至2023年12月。结果87例患者中，女性50例，男性37例，平均年龄为（52.3 ± 14.2）岁（17-91岁）。58.6%（n = 51）的患者患有颈动脉体肿瘤，25.2%（n = 22）的患者患有迷走神经胶质瘤，6.8%（n = 6）的患者患有中耳肿瘤，2.2%（n = 2）的患者患有咽旁间隙肿瘤，1.1%（n = 1）的患者患有鼻窦肿瘤。5.7%的患者（5人）患有多灶性疾病。发病时肿瘤的平均大小为 3.2 ± 1.4 厘米（0.5-6.9 厘米不等）。41.3%的患者（n = 36）、12.6%的患者（n = 11）、2.2%的患者（n = 2）和1.1%的患者（n = 1）发现了致病性SDHD突变、SDHB突变、SDHC突变和SDHA突变。总体治疗方式包括：51.7%（45 例）的患者接受单纯手术治疗，14.9%（13 例）的患者接受放射治疗，28.7%（25 例）的患者接受观察治疗，4.5%（4 例）的患者接受使用奥曲肽的体泌激素类似物治疗。复发风险明显较高的相关因素包括年龄超过60岁（p = .04）、肿瘤大小超过2厘米（p = .03）、SDHx变异阳性（p = .01）以及迷走神经和颈静脉肿瘤（p = .04）。我们的研究结果表明，只要坚持终生监测，经过精心挑选的无症状或身体状况不佳的患者可以得到安全的观察。我们主张建立英国和爱尔兰全国 HNPGL 登记处，以确定这些罕见肿瘤的最佳治疗策略，改善长期预后。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Clinical Endocrinology 医学-内分泌学与代谢

CiteScore

6.40

自引率

3.10%

发文量

192

审稿时长

1 months

期刊介绍： Clinical Endocrinology publishes papers and reviews which focus on the clinical aspects of endocrinology, including the clinical application of molecular endocrinology. It does not publish papers relating directly to diabetes care and clinical management. It features reviews, original papers, commentaries, correspondence and Clinical Questions. Clinical Endocrinology is essential reading not only for those engaged in endocrinological research but also for those involved primarily in clinical practice.