Endocrine Abstracts

JOINT3347

Introduction: Phaeochromocytomas, Paragangliomas, Glomus jugulare are rare neuroendocrine tumours arising from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia. Most of them produce catecholamines. They cause variable symptoms like headache, hyperhidrosis, tachycardia, and hypertension.

Case: A thirty seven year old window cleaner, was referred to Cardiology with a six months history of worsening random dizziness, impacting on his work. He reported one to five minutes episodes of feeling faint, chest tightness, breathlessness, closing of his throat, tingling sensation of the face, grey face and hands. Symptoms occurred up-to five times per day. He has a ten year history of random palpitations, dizziness and non-cardiac chest pain, which was evaluated by Cardiology. Six months previously, he was commenced on a diet for hypercholesterolemia. He takes no medications or supplements. He binge drinks, smokes nicotine, uses cocaine once or twice per week and used cannabis previously. No family history of note, except father dying at age of forty six from a heart attack. Clinic blood pressure was one hundred and thirty one over ninety three mmHg. He had two symptomatic episodes of his blood pressure raising to two hundred and forty one over one hundred and seventeen mmHg for two and then three minutes. His physical examination was unremarkable. ECG and Holter were normal. Echocardiogram revealed mild concentric LVH, preserved LVEF. Renal function, electrolytes, cortisol, TSH, CK, aldosterone/renin ratio were normal. Urine metanephrines were raised. Doxazosin initiated. He underwent adrenal MRI revealing a large left adrenal tumour suggestive of phaeochromocytoma. Contrast CT adrenal, abdomen, NM MIBG whole body were consistent with beta-adrenergic receptor positive tumour tissue of neural crest in origin in the left adrenal gland, most probably due to a solitary phaeochromocytoma. He was referred to Endocrine MDT and admitted for a laparoscopic left adrenalectomy. The histology result was consistent with benign pathology and a follow-up was arranged for a repeat metanephrine +/- MIBG or MRI if positive metanephrines.

Discussion: Thorough evaluation led to treating this patient’s symptoms and preventing cardiovascular, respiratory, metabolic or neurological complications including death.

Conclusion: Phaeochromocytomas, Paragangliomas, Glomus jugulare tumours cause less than zero point one percent of the hypertension cases. It is extremely important to exclude these conditions as a cause for hypertension in male and female patients in their third and forth decade because of a high risk of death. The post-mortem diagnosis is as high as fifty percent. Managing phaeochromocytomas require multidisciplinary approach.