Endocrine Abstracts

JOINT3756

Phaeochromocytoma/paragangliomas (PPGLs) are relatively rare tumours and the health burden of such tumours is not very well known.

Aim: This population-based study aims to characterise all the phaeochromocytomas, paragangliomas and adrenal medullary hyperplasia diagnosed between 2010 and 2023 in Malta; looking into presentation, hormonal analysis, imaging characteristics and histology findings.

Results: 29 patients were identified (22 phaeochromocytoma and 7 paragangliomas). 16 patients (55%) were males and age ranged from 21-81 years (median (IQR) 53.5 years (40-59.8)). The standardised incidence rate (SIR) was 0.35/100,000/year. The SIR for phaeochromocytoma was 0.27/100,000/year and that for paraganglioma was 0.09/100,000/year. Longest radiological tumour size ranged from 20-127mm (median (IQR) 51mm (33.5-65)). Median pre-contrast CT density was 34 Hounsfield units (IQR: 29.5-34.5). All patients except 2 underwent surgical resection of the tumours. The latter 2 patients presented late with metastasis and died soon after diagnosis. Genetic testing was done in the majority of patients and VHL, NF1 and SDHB mutations identified. 6 patients (20%) were found to have a malignant PPGL on follow up.

Conclusion: This study gives a detailed understanding of the epidemiology of PPGL in a very well-defined population. The significant rate of malignant disease emphasizes the need for long-term follow-up.