Endocrine Abstracts

Background: Most phaeochromocytomas are unilateral and are not associated with a neuroendocrine syndrome. Although these tumours are often histologically benign, they tend to have potentially lethal presentations.

Case: A 51-year-old female presented in 2016 with headache, vomiting, palpitations, and a cardiac-sounding chest pain for 16 hours. Her ECG showed a junctional rhythm with generalised ST-segment depression. Acute coronary syndrome treatment was commenced. Her symptoms had been intermittent and progressive over 14 months prior to this presentation. On arrival, her blood pressure (BP) was 137/70 mmhg and her heart rate (HR) was 92/min. Whilst in ED; she suddenly deteriorated with worsening breathlessness and escalating chest pain which radiated to her back with a sudden rise of her BP to 250/140 mmhg and HR to 160/min. O2 Saturations were 78% on 15 L oxygen via facemask. She developed ventricular fibrillation and required immediate cardiopulmonary resuscitation. The patient was intubated and transferred to intensive care unit. Glyceryl trinitrate infusion was administered to control her BP. A CT Aortogram showed a 7 cm right adrenal mass, compressing the inferior vena cava. A bedside echocardiogram showed poor contractibility of the left ventricle. The patient developed acute kidney injury and her liver functions were deranged. N-Acetyl Cysteine infusion was commenced. Plasma normetanephrines were 10672 pmol/l and metanephrines were 6885 pmol/l. MIBG scan confirmed right pheochromocytoma diagnosis. She was on intravenous anti-hypertensive treatment (Phentolamine and esmolol) which were later switched to Doxazosin and Bisoprolol. Once stable, the patient underwent laparoscopic resection of her right adrenal pheochromocytoma with uneventful recovery. Genetic testing for pheochromocytomas/paragangliomas (PPGL) was negative. Histopathology confirmed a pheochromocytoma diagnosis with no vascular or capsular invasion or extension into the adjacent fat. The PASS score was 7. And the Ki67 index was approximately 1%. Since the curative surgery, the patient’s kidney, liver and heart failures were reversed back to normal. She remains under regular follow up and all of her subsequent post-operative metanephrines and nor-metanephrines are undetectable and is not requiring any anti-hypertensive treatment now.

Discussion: Phaeochromocytoma is classically associated with periodic hypertension and intermittent and variable symptoms like headache, sweating, and palpitations which are difficult to diagnose particularly in females at this age group. These symptoms are attributed to the excessive release of catecholamines from the tumour’s chromaffin tissue. Management should focus on initial stabilisation of the disturbed haemodynamic status, followed by adequate pre-surgical α-blockade and β-blockade, if indicated and deemed necessary. Urgent surgical intervention tends to be associated with high surgical morbidity and mortality.

Conclusion: Despite most phaeochromocytoma tumours tend to be histologically benign, they may lead to serious life-threatening complications. However, the disease is curable if detected early and the tumour is resected with appropriate presurgical medical therapy. Finally, pheochromocytoma confirmation warrants further investigations to exclude a multiple endocrine disorder (e.g. MEN-2a)