Endocrine Abstracts

Background: Paragangliomas are rare neuroendocrine tumours of the sympathetic and parasympathetic paraganglia. Optimal outcomes are dependent on careful investigation, management and follow-up.

Aim: The aim of this audit was to review clinicopathological characteristics of a cohort of operated abdominal paragangliomas in Northern Ireland.

Methods: This is a retrospective audit of abdominal paragangliomas from the Northern Ireland regional service operated 01/01/2014-31/12/2023. Cases were identified by searches of SNOMED codes in histopathological archives. Clinical and laboratory data were collected from retrospective electronic chart review.

Results: A total of 26 patients were operated for abdominal paraganglioma. Twelve (47%) patients were female, median age 55 years (range 18-72). At presentation 10/19 (53%) had documented classical symptoms of headache, sweating or palpitations and 5/20 (25%) were detected as incidentalomas. There was a formal diagnosis of hypertension in 15/23 (65%) pre-operatively and 15/20 (75%) had preoperative evidence of functionality (elevated urine or plasma normetanephrines or metanephrines ≥2x upper limit of reference range requiring pre-operative alpha blockade). Six patients not tested pre-operatively were diagnosed intraoperatively or postoperatively on histological assessment. Median radiologically reported maximum tumour diameter was 49mm (range 20-185). Genetic testing results were available in 22 patients, of whom 12 (55%) had SDHA, SDHB, SDHC or VHL variants. Median follow up was 40.2 (range 0.2-109.7) months. Two patients had metastatic paraganglioma and one had synchronous paraganglioma and phaeochromocytoma at diagnosis. Metastatic disease management included surgery, somatostatin receptor ligand, tyrosine kinase inhibitor, stereotactic radiotherapy and peptide receptor radionuclide therapy. Three patients were deceased at follow up.

Conclusions: Multidisciplinary care of abdominal paraganglioma in Northern Ireland demonstrates good clinical outcomes. In this cohort, albeit modest follow up, there was no local recurrence. Future work will expand this dataset and utilise it to continue enhancing the care of patients living with paragangliomas in the region.