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

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

doi:10.1111/cen.15070

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

Clin Endocrinol (Oxf). 2024 May 2. doi: 10.1111/cen.15070. Online ahead of print.

Authors

Milad Darrat¹, Louis Lau², Colin Leonard³, Stephen Cooke⁴, Muhammad A Shahzad¹, Claire McHenry¹, David R McCance¹, Steven J Hunter¹, Karen Mullan¹, John R Lindsay¹, Una Graham¹, Neil Bailie³, Susie Hampton³, Simon Rajendran⁵, Fionnuala Houghton⁶, David Conkey⁶, Patrick J Morrison⁷, Philip C Johnston¹

Affiliations

¹ Regional Centre for Endocrinology and Diabetes, Royal Victoria Hospital, Belfast, UK.
² Department of Vascular Surgery, Royal Victoria Hospital, Belfast, UK.
³ Department of Otolaryngology, Royal Victoria Hospital, Belfast, UK.
⁴ Department of Neurosurgery, Royal Victoria Hospital, Belfast, UK.
⁵ Department of Pathology, Royal Victoria Hospital, Belfast, UK.
⁶ Department of Oncology, Belfast City Hospital, Belfast, UK.
⁷ Department of Clinical Genetics, Belfast City Hospital, Belfast, UK.

PMID: 38696538
DOI: 10.1111/cen.15070

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.

Keywords: clinical management; head and neck paragangliomas; outcomes; recurrence.