An Atypical Presentation, Complication and Preoperative Management of Pheochromocytoma

Presentation Number: SAT 387
Date of Presentation: April 1st, 2017

Franco Alejandro Vallejo*, Carolina Hurtado, Katherine Lopez and Rodrigo Garcia
Icahn School of Medicine at Mount Sinai - Mount Sinai West & Mount Sinai St Luke's, New York, NY

Abstract

INTRODUCTION

Pheochromocytoma is a rare tumor from chromaffin cells of the adrenal medulla first described in 1884 by Felix Fränkel. This tumor has an annual incidence of 0.8 per 100,000 person year.

CASE PRESENTATION

A 56-year-old Caucasian man went to the emergency room because of sudden left lower extremity pain radiated to the chest and back. The patient’s physical examination showed blood pressure (BP) of 110/70, heart rate of 112 bpm and diaphoresis, the rest was unremarkable. The EKG did not show signs of ischemia although troponin was elevated. Patient was sent to coronary catheterization which revealed normal coronaries and severe left ventricular dysfunction and ejection fraction (EF) of 20%. Twelve hours after the procedure the patient developed hypotension and shock requiring vasopressor support. Hemoglobin dropped from 15 g/dl to 9.4 g/dl. CT abdomen was done to rule out bleed and showed a large left retroperitoneal hematoma and a retroperitoneal mass (12x10x13 cm) displacing the kidney into the pelvis. Patient's clinical course progressed to severe acute kidney injury and anuria requiring temporal hemodialysis. An embolization of the left middle adrenal artery was done to stop the bleeding. Endocrine work-up of retroperitoneal mass resulted in normal values of 7-OH progesterone, androstenedione, DHEA sulfate, testosterone and cortisol. Urine and plasma metanephrines were consistently elevated in multiple occasions and the diagnosis of pheochromocytoma was made.

For preoperative treatment patient was started on alpha blocker (doxazosin 1mg daily) but patient had multiple episodes of hypotension. Given intolerance to alpha blocker pre-operative treatment consisted mainly of metyrosine 250mg every 6 hours before surgery. After 14 days of pre-op treatment he was taken to surgery to remove the mass. The pathology showed a benign pheochromocytoma. Patient improved clinically and his EF increased to 60%.

DISCUSSION

This case highlights a unique presentation and complication of pheochromocytoma and opens the possibility of different treatment modalities when patients cannot tolerate alpha blocker. Our patient presented with lower extremity and back pain with retroperitoneal hematoma and did not have any of the classic symptoms of episodic headache, sweating, tachycardia and hypertension. About 5-15% of patients can present with normal blood pressure like is the case with our patient. He also had a rare complication of pheochromocytoma which is dilated cardiomyopathy and it is due to catecholamine production by the tumor. Lastly our patient was not able to be treated with alpha blocker given recurrent episodes of hypotension so treatment was started with metyrosine which inhibits the production of catecholamines and is an uncommon treatment modality.

 

Nothing to Disclose: FAV, CH, KL, RG