The Challenge of a Great mimic: Case report of Pheochromocytoma presenting as Acute Coronary Syndrome complicated by Retroperitoneal bleed and Multisystem Crisis.
Rumaisa Banatwalla (Endocrinology Registrar, ST4), Victoria Hodge (GPST2, Cardiology), Helen Ward (Endocrine Consultant & Clinical Lead), Gautam Das (Endocrine Consultant), Thomas FitzGerald (FY1 Endocrine), Tahir Ali (Consultant Vascular, Endovascular and General Surgeon), Anthony Parsons (ITU Consultant), St Peters Hospital, Chertsey.
Pheochromocytomas are catecholamine secreting tumours of the adrenal medulla. They are present in 0.1-0.6% in hypertensive patients1.10-30% are incidentally discovered by imaging2. A 50 year autopsy review of 40 000 cases, showed 76% of Pheochromocytomas were only identified after death3. Pheochromocytoma crisis, is a potentially lethal complication of Pheochromocytoma4. Management includes initial stabilization of the acute crisis followed by surgery5.
Case: This 81 year old man was under investigation for a Right Adrenal lesion. He had episodes of labile blood pressure at home and presented to the Accident Emergency with Pheochromocytoma crisis mimicking Acute Coronary Syndrome. On arrival to the hospital, he was treated with dual antiplatelet agents and Coronary Angiogram showed Triple Vessel Disease with normal LV function. Doxazosin 1mg was stopped on arrival and was commenced on a Beta blocker. Next day he experienced cyclical episodes of hypertension and hypotension with clinical signs of peritonism. He underwent a CT Abdomen which revealed a massive retroperitoneal bleed and enlarged right adrenal gland. He was stabilised with phentolamine, fluid resuscitation, and blood transfusion, followed by an emergency laparotomy. He died 40 days later after a prolonged ITU stay complicated with Metabolic Encephalopathy, Klebsiella Sepsis and Hospital Acquired Pneumonia.
Discussion: Diagnosis of Pheochromocytoma is based on a combination of clinical findings and relevant tests. There is a high mortality of 28% following emergency resection of Pheochromocytoma2,6 .Pheochromocytomas can present with a multi-system crisis7. Some of these features were observed in our patient.
Conclusion: Early recognition and prevention is the key, particularly given the high mortality for patients undergoing emergency resection of Pheochromocytoma.
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2. Souiki T et al. World Journal of Emergency Surgery 2014, 9:50
3. Fahmy WF Hanna et al. Management of incidental adrenal tumours. BMJ 2018:360:j5674
4. Riester A et al. European Journal of Endocrinology 2015; 173(6),757-764
5. Scholten A et al. Journal of Endocrine Metab 2013; 98:581-591
6. Kobayashi T et al. J. Surg Oncol. 2005; Apr 1, 90(1):31-35
7. Mitsis A et al. Hellenic Society of Cardiology 2017;58, 372-377