To investigate the impact of a medication called Belzutifan on the production and subsequent metabolism of adrenaline and noradrenaline collectively termed 'catecholamines'. The study aims to identify changes in the production and metabolism of catecholamines by measuring the substance which starts the chain of catecholamine metabolism called tyrosine in patients before, during and after 5 days of taking Belzutifan 120mg daily.
Adult patients > 18 years
Patients must have a biochemically confirmed diagnosis of a phaeochromocytoma or paraganglioma using plasma metanephrines or 24- hour urinary metanephrines and plasma or urinary metanephrines should be at least 1.5 times the upper limit of the normal reference range.
Female patients of child-bearing potential must have a negative serum pregnancy test result within 3 days before first administration of study drug
Able to provide informed consent
Has hypoxia, or requires intermittent supplemental oxygen, or requires chronic supplemental oxygen.
Has clinically significant cardiac disease, including unstable angina, acute myocardial infarction, percutaneous transluminal coronary angioplasty (PTCA), or coronary artery bypass graft surgery (CABG) ≤6 months from Day 1 of study drug administration, or New York Heart Association Class III or IV congestive heart failure. Medically controlled arrhythmia stable on medication is permitted.
Has a co-existing malignancy (in addition to PPGL)
Has a Hb < 100g/dL
Cambridge University Hospitals NHS Foundation Trust
Lead sponsor
University of Cambridge
Collaborator