690 Paragangliomas: A Rare and Under-Appreciated Aetiology? a Case Series

Aim To assess the presentation and management of paragangliomas within NHS Grampian between 2018 to 2023. Method Retrospective data collection from electronic records of histo-pathologically confirmed paragangliomas for: mode of presentation (incidental vs symptomatic), location of tumour, biochemistry (urinary/ plasma metanephrines), management, genetics, follow-up, and outcome. Results Ten cases of paragangliomas were identified, with an age range of 17-76 years: two head and neck, one thoracic, six abdominal and one pelvic. Seven presented with symptoms. Five had elevated metanephrines, though these were only measured in four cases pre-operatively. Surgical excision was undertaken in six. Chemotherapy given in three. Nine were discussed in multidisciplinary (MDT) meeting. Six patients in remission with ongoing surveillance, three have persistent / recurrent disease, and one is deceased. Pathogenic Succinate Dehydrogenase germline variant present in three, no genetic variance in five, results pending in one and one unknown. Conclusions Paragangliomas are rare neuroendocrine tumours that may present to a diverse group of surgical specialties. They occur in any age group, have a high incidence of malignancy and germline genetic mutations; and should be suspected in an unexplained mass arising along the sympathetic chain. Biochemical evaluation should be assessed for metanephrine excess and alpha receptor blockade should be undertaken prior to invasive interventions in functional tumours to mitigate risk of provoking an adrenergic storm. Paragangliomas need discussion in endocrine/ neuroendocrine tumour MDT to ensure appropriate pre-operative workup and follow up. This may improve outcomes and aid in the earlier detection of recurrent disease.