Two patients have presented to a regional Australian Hospital with intrapericardial paragangliomas in the last decade. In 2011, a 28-year-old Indigenous male presented with a hypertensive crisis. He had a two-year history of symptoms during physical exertion, including abdominal pain, nausea, and vomiting. He had also experienced paroxysmal light-headedness and sweats, and had been previously diagnosed with hypertension. Urinary and serum catecholamine testing revealed markedly elevated noradrenaline. Subsequent imaging showed a synchronous functioning paraganglioma in the right atrium and retrocaval region. He had a surgical resection of both lesions, with normalisation of catecholamines post-operatively. The SDHB gene mutation was identified, however the ongoing follow-up of this patient has since been limited. The second case, a 38-year-old female, presented in 2018 with a 12-month history of paroxysmal severe headaches, flushing, tachycardia and eight kilograms of weight loss. Workup revealed significantly elevated urine and plasma fractionated metanephrines, including noradrenaline, dopamine, vanillylmandelic acid, normetanephrine, and 3 methoxytyramine. Gallium-68 DOTATE PET revealed an intensely DOTATATE avid left atrial/atrial-septum lesion consistent with paraganglioma. Further imaging included a cardiac MRI and echocardiogram. She 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 70 mm tumour in the inter-atrial groove. Histopathology was consistent with paraganglioma and her genetic studies showed SDHC mutation.
18 - 21 May 2019
European Society of Endocrinology