Headaches from the heart (#216)
Hannah Farquhar 1 , Gaurav Puri 1
Cairns hospital, Cairns North, QLD, Australia
Ms PI, a 38 year old female living in a rural town, presented with a 12 month history of paroxysmal severe headaches, flushing, tachycardia and unintentional weight loss of eight kilograms. She had seen multiple different GP's over this time, and had been diagnosed with migraines and psychological issues. Endocrine was consulted upon review with a new GP. Upon Endocrine review, Ms PI was not hypertensive, and examination was unremarkable. She had no personal or family medical history. Work-up revealed significantly elevated urine fractionated metanephrines and catecholamines, including noradrenaline, dopamine, vanillylmandelic acid, normetanephrine, and 3 methoxytyramine. This correlated with raised plasma metanephrines, and so work-up proceeded to localise the source of her excess catecholamines. Initial MRI abdomen was unremarkable, thus she went on to have a Gallium-68 DOTATE PET, which revealed an intensely DOTATATE avid left atrial/inter-atrial septum lesion consistent with neuroendocrine tumour/paraganglioma. Further imaging included a cardiac MRI and echocardiogram. Ms PI underwent pre-operative assessment with a coronary angiogram, which showed 3 coronary artery tributaries feeding the tumour. She was pre-operatively treated with phenoxybenzamine, followed by metoprolol. She had a surgical resection which revealed a highly vascular 70mm tumour in the inter-atrial groove. Histopathology was consistent with paraganglioma and her genetic studies are pending. Cardiac paraganglioma's are exceptionally rare, and this case was the second case in our centre over the past decade. Her case highlights the importance of considering a pathological cause for all systemic symptoms, and that excellent and prompt specialist care can still be delivered despite living in a rural location. Her case also demonstrates the utility of DOTATATE PET in localising paragangliomas.