Case report of patient with pheochromocytoma presenting with gangrene and Diabetes

Abstract

INTRODUCTION :A pheochromocytoma is a rare, catecholamine-secreting tumour that may precipitate life-threatening hypertension. The tumour is malignant in 10% of cases but may be cured completely by surgical removal. Because of excessiv e catecholamine secretion, pheochromocytomas may precipitate life-threatening hypertension or cardiac arrhythmias. If the diagnosis of a pheochromocytoma is overlooked, the consequences can be disastrous, even fatal; the diagnosis can be established by measuring catecholamines and metanephrines in plasma (blood) or through a 24-hour urine collection. The most common clinical sign of pheochromocytoma i s sustained or paroxysmal hypertension, and the most common symptoms are headache, excessive truncal sweating, and palpitation. In some cases, the clinical symptoms are not clear. Roughly 70% of adrenal incidentalomas are non-functional. A small group of 5–7% of the functional ones (30%) may exist as pheochromocytoma. Ten percent of pheochromocytoma cases are diagnosed incidentally during computed tomography (CT) or magnetic resonance imaging (MRI) screenings for other reason s. CASE PRESENTATION : 21 year old female patient who was referred to Kenyatta National Hospital with diagnosis of gangrene in a young lady newly diagnosed with diabetes in a known hypertensive for three years. The gangrene was of a duration of two weeks. She was diagnosed with diabetes during work up for the cause of the gangrene. Investigations revealed a 24 hour-urine norepinephrine levels of 5085nmol, Normetanephrines excretion of 45213nmol over 24hoursShe tested negative for HIV, Hepatitis B and C surface antigens, VDRL, ANA, C-Anca and P-A nca. Abdominal ultrasound showed normal sized kidneys with a suprarenal mass (80 *63) mm with ectopic right kidney in pelvis, ECG a sinus tachycardia, Echo cardiogram reported as normal wit h an LVEF of 54%. Arteriogram had a vasoocclusive disorder at the digital femoral artery an d CT abdomen showing a supra renal mass (8x6x5) cm border of head and body of pancreas displacing the right kidney inferiorly. The patient underwent an amputation of the limb and adrenelectomy. Following the surgery the blood pressure and the glucose has normalised and currently is of medication CONCLUSION: Diagnosis of hypertension in a young patient should involve looking for secondary causes of the disease. A young hypertensive patient presenting with a triad of headaches, palpitations, and sweating was then investigated for pheochromocytoma. Pheochromocytoma can present and occur as an emergency ranging from pheochromocytoma-related multisystem failure, cardiovascular emergencies, pulmonary emergencies, abdominal emergencies, neurologic emergencies, renal emergencies, and metabolic emergencies. These presentations are associated with a high morbidity and mortality if pheochromocytoma is unsuspected. This presentation was unique because it was none of the expected emergencies but a rapidly evolving asymmetrical gangrene of the right foot.