Pheochromocytoma Presenting With Cardiomyopathy in an Otherwise Healthy Young Patient: A Rare Case Report

Abstract

Background: Pheochromocytomas are rare neuroendocrine tumors. The classic triad of symptoms includes diaphoresis, episodic headache, and tachycardia. Rarely, patients may develop cardiomyopathy associated with pheochromocytoma. It is uncommon for cardiomyopathy to be the initial presenting symptom of pheochromocytoma and can lead to a delayed diagnosis.Clinical Case: The patient is a 30-year old female with no significant medical history who presented with complaints of dyspnea, fever and malaise. The patient had no known prior history of hypertension and did not report complaints of headache, diaphoresis or palpitations. Patient was hypertensive and tachycardic on admission. Upon treatment with intravenous fluids, the patient acutely decompensated and developed respiratory failure requiring emergent intubation. CT of the chest, abdomen and pelvis was performed which showed diffuse bilateral pulmonary edema as well as a circumscribed, heterogeneously enhancing 4.4 cm mass involving the right adrenal gland. An echocardiogram was performed which showed significant left ventricular dysfunction with ejection fraction of 5%. Patient had wide fluctuations in her blood pressure during admission ranging from hypotension to extreme hypertension. She did require the use of epinephrine and milrinone when hypotensive. Due to the use of vasopressors, plasma fractionated metanephrines and 24-hour urine fractionated metanephrines and catecholamines were not recommended to be obtained as these medications may contribute to false positive testing. A dedicated CT of the abdomen with and without contrast was performed to further assess the patient’s right adrenal mass; this revealed a 3.9 x 2.4 x 4.9 cm right adrenal mass with 33% absolute washout and 17% relative washout concerning for pheochromocytoma. The patient underwent an open right adrenalectomy during admission. Surgical pathology results were consistent with a pheochromocytoma measuring 5.2 cm at greatest dimension; the tumor was confined to the adrenal gland and completely excised. Following her surgery, the patient’s clinical status improved significantly over the next several days. She was discharged home in stable condition on hospital day #18. An echocardiogram was repeated 2 months after her hospitalization with ejection fraction improved to 58%.Conclusion: Catecholamine-induced cardiomyopathy in pheochromocytoma is rare, occurring in approximately 8–11% of patients with pheochromocytoma. Cardiomyopathy as the initial presentation of pheochromocytoma is very uncommon. It is important to accurately diagnose pheochromocytoma-related cardiomyopathy as early detection and resection can lead to marked improvement in cardiac function and prevent catastrophic consequences including death.