SFEBES2023 Poster Presentations Neuroendocrinology and Pituitary (74 abstracts)
Phaeochromocytoma/Paraganglioma – Call for Education and More Screening
Alwyn Yung Zhuang Choo 1 , Rupa Ahluwalia 1 , Neetha Joseph 1 , Janak Saada 1 , Nicholas McArdle 1 , Allison Chipchase 1 , Mark Rochester 1 , David Manson-Bahr 2,1 , Melanie Maxwell 1 & Khin Swe Myint 1,3
1Norfolk and Norwich University Hospital, Norwich, United Kingdom. 2James Paget University Hospital, Great Yarmouth, United Kingdom. 3Norwich Medical School, University of East Anglia, Norwich, United Kingdom
Background: Phaeochromocytomas & paragangliomas (PPGL) are rare neuroendocrine tumours presenting with symptoms of sympathetic overactivity, hypertension, or as adrenal incidentalomas.
Study and results: A 24-year retrospective review of a U.K. single-centre PPGL service (2009 -2023) identified 61 cases (53 phaeochromocytomas/8 paragangliomas). Among these, 35 (57%) were females. Mean age of diagnosis was 56.35±16.2 (SD) years. Only 13 (21%) presented with symptoms and were screened, while 48 (79%) were incidental findings. Of those incidental, 8 (17%) had hypertension/cardiovascular disease, 15 (31%) had phaeochromocytoma symptoms, and 13 (27%) had both. 95% had raised metanephrines at diagnosis and lesion identified with CT/MRI (mean size 42.5mm, range 8-90). MIBG scans were undertaken in 52 cases (85%) with increased uptake in 47 (92%) including metastatic disease identified in three cases. Diagnosis was made through histology in three cases following intervention for other reasons. Genetic screening was performed in 38 (62%) cases, with 13 identified to have mutations associated with PPGLs. Six cases (46%, n=13) had neurofibromatosis type 1 (NF1), all presenting as adrenal incidentalomas. All biochemically active cases were treated with alpha-blockers. 53 patients underwent surgery (89% laparoscopically), two are awaiting surgery, six were treated conservatively (five unfit for surgery, one declined). Perioperative blood pressure stability was observed in 52 patients, with no mortalities. Four patients experienced recurrence, and one had metastasis after surgery. Post-surgery, patients exhibited a mean weight gain of 3.3±6.02 (SD) kg, likely due to reduced sympathetic activity and basal metabolic rate.
Conclusion: The study highlights the increasing diagnosis of PPGLs through incidentaloma screening. Missed symptoms were prevalent in 70% of incidental cases. NF1 patients comprised 46% of those with positive gene mutations, emphasising the need for phaeochromocytoma screening in this population. MIBG imaging was valuable for identifying active lesions, and thorough preoperative optimisation was crucial for favourable outcomes.
Volume 94
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Endocrine Abstracts
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