SFEBES2026 Oral Communications Mechanisms and Management of Endocrine Disease (5 abstracts)
Retrospective Review of the Management of Head and Neck Paragangliomas in a UK PPGL Centre of Excellence
William Jackson 1 , Juliette Buttimore 2 , Danilo Inchiappa 2 , Victoria Stokes 2 , Ben Challis 2 , Sarah Jefferies 2 , Katherine Burton 2 , Soo-Mi Park 2 , Sarah Wilcox 2 , Daniele Borsetto 2 , Brian Fish 2 , Simon Kreckler 2 , James Tysome 2 , Tilak Das 2 , Daniel Scoffings 2 & Ruth Casey 2
1University of Cambridge, Cambridge, United Kingdom; 2Cambridge University Hospitals, Cambridge, United Kingdom
Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumours predominately arising from parasympathetic ganglia. HNPGLs can be sporadic or hereditary, rarely secrete catecholamines and have malignant potential. Herein we report a series of HNPGLs in a UK tertiary referral centre. A retrospective review of 79 patients (50 female) diagnosed between 2014-2025 was performed. Patients were excluded from data analysis if data sets were incomplete. The cohort’s median age was 58 years (range 20-86). Tumour location was jugulotympanic for 65.8% (52/79), carotid body for 17.7% (14/79), vagal for 6.3% (5/79), multifocal within the head and neck in 8.8% (7/79) and one patient had a HNPGL and a synchronous abdominal paraganglioma. The median tumour size from radiological reports was 23x19mm. A pathogenic variant in the succinate dehydrogenase (SDHx) genes was identified in 38% (30/79) of these: 60% (18/30) SDHB, 30% (9/30) SDHD, 6.7% (2/30) SDHA and 3.3% (1) SDHC. Plasma metanephrines or plasma 3-methoxytyramine were elevated in 20.3% of cases (16/79). Of those with raised 3-methoxytyramine: 75% (9/12) had SDHx mutations (6/9 SDHB). Of the SDHx carriers, 70% (21/30) were probands and 30% (9/30) were gene carriers identified through screening programmes. Management included surgical excision in 18/79 (22.8%), surgery and radiotherapy in 3/79 (3.8%), radiotherapy alone in 19/79 (24.1%) and surveillance in 39/79 (49.4%). 11/79 (13.9%) patients developed local recurrence of their tumour or tumour growth. Of these: 36.4% (4/11) had local regrowth after partial excision ± radiotherapy treatment and 63.6% (7/11) had local recurrence after total surgical excision. 10/12 (83.3%) patients who developed recurrent or progressive growth of HNPGLs harboured an SDHx mutation. This series produced results comparable to other reported cohorts with a ~15% rate of recurrent or progressive HNPGL growth and a similar prevalence of SDHx mutations and highlights the need for long term follow up and expert MDT input.
Volume 117
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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