SFEEU2018 Clinical Update Workshop E: Disorders of the adrenal gland (17 abstracts)
Darent Valley Hospital, Dartford, UK.
A 64 year old female with a background history of hypertension resistant to dual therapy had recurrent admissions to the local emergency department with pulmonary oedema and chest tightness over the preceding 3 months associated with fluctuations in Troponin I levels. This led to multiple cardiac investigations including invasive coronary angiogram which did not report any abnormality. Her blood pressure was elevated at 200/120 mmHg and an electrocardiogram demonstrated sinus tachycardia on return of her chest pain. Investigation with a CT chest, abdomen and pelvis for ongoing back pain revealed a possibility of an incidental 3.5 cm right adrenal adenoma. Significantly elevated 24 hour urinary catecholamines confirmed the diagnosis of phaeochromocytoma (24 hour urinary metadrenaline of 13.19 umol/24 hour (normal reference range <1.20), 24 hour urinary normetadrenaline 10.89 umol/24 hour (normal reference range <3.30), total metadrenalines 24.08 umol/24 hour (normal reference range <4.50)). The MIBG scan correlated with the findings; demonstrating extensive uptake within the mass. Following referral to the tertiary centre she underwent an open right adrenalectomy with normal post-operative levels of catecholamines and remains asymptomatic with a blood pressure of 133/80 mmHg on annual reviews and has discontinued all of her anti-hypertensive medications. There are important considerations when evaluating resistant and unusual cases of hypertension. Electrocardiogram changes with phaeochromocytoma are common and up to 12% of patients will present with coronary syndromes. Elevated cardiac enzymes can be a prognostic indicator for those at risk of developing myocardial damage. It is therefore an important consideration when managing patients with chest pain including those with ischaemic ECGs. The general prevalence of phaeochromocytoma in people diagnosed with hypertension is around 0.5% and this increases to 1.7% in the paediatric population. Less than 5% of incidental adrenal lesions prove to be phaeochromocytoma. Of the cases identified approximately 30% will have a genetic predisposition which may alter treatment and surveillance. This patient did not have any features suggestive of syndromic association including multiple endocrine neoplasia, Von Hippel Lindau syndrome or neurofibromatosis however the case raises the importance of considering when and who to screen for genetic testing with a confirmed phaeochromocytoma.