Giant Adrenal Myelolipoma Masquerading as Heart Failure
A 52-year-old man with a past medical history significant for atrial fibrillation, diabetes type 2 and hypertension for 30 years was admitted with early satiety for the past 3 months and progressive dyspnea on exertion for 3 weeks. He also complained of gradual unintentional weight loss of approximately 100 pounds over the last 10 years. He had been a non-smoker and a moderate alcohol user for the last 30 years. On initial presentation to the hospital, he denied shortness of breath at rest or chest discomfort and was in no acute distress.\nHis blood pressure was 204/93 mm Hg on the left arm and 194/88 mm Hg on the right arm with an irregular heart rate of 54 beats/min and oxygen saturation of 96% on room air. The lungs were clear to auscultation, jugular venous distension was noted at 13 cm above the sternal angle and 3+ bilateral leg edema with scrotal edema. Cardiac auscultation was pertinent for 3/6 systolic ejection murmur over the left sternal border. His abdomen felt firm and lacking in tympanicity, but it was non-tender in all four quadrants. Bowel sounds were audible but faint. Laboratory values were notable for a brain natriuretic peptide of 980 ng/l, creatinine of 1.2 mg/dl, BUN of 20 mg/dl, HCO3 of 31 mEq/l, INR of 1.23, Hb of 12.5 g/dl, Na of 140 mEq/l, K of 3.7 mEq/l and negative cardiac troponins. He was monitored in the Intensive Care Unit for decompensated heart failure with uncontrolled hypertension.\nTo promote diuresis and reduce afterload, he was administered intravenous furosemide 80 mg, oral metolazone 2.5 mg, oral hydralazine 75 mg every 6 h, oral clonidine 0.3 mg, lisinopril 40 mg, isosorbide mononitrate 120 mg and losartan 100 mg. Overnight, he had an episode of moderate self-limited epistaxis with minimal improvement in blood pressure control. He remained in slow atrial fibrillation with a lowest recorded heart rate of 38 beats/min. However, he denied dizziness at any time despite a negative fluid balance of 1.8 liters. Renal function worsened with an increase of creatinine to 1.6 mg/dl.\nAn echocardiogram showed a left ventricular ejection fraction of 30% with four-chamber dilatation, moderately reduced right ventricular systolic function, severe mitral regurgitation and moderate pulmonary hypertension. He underwent right heart catheterization showing a right atrial pressure of 12 mm Hg, right ventricular pressure (systolic/diastolic) of 60/12 mm Hg, pulmonary artery pressure of 60/23 mm Hg and pulmonary capillary wedge pressure of 20 mm Hg and a cardiac index of 2.3 liters/min. A left heart catheterization revealed clean coronaries, and alcohol was thought to be the likely etiology of non-ischemic cardiomyopathy in this patient. Urine catecholamine and vanillylmandelic acid were measured and a magnetic resonance angiography of the renal arteries was performed to evaluate for secondary causes of hypertension. This showed non-obstructed renal arteries bilaterally.\nA minimally enhancing 22 × 16 × 23 cm mass was visualized in the left upper and mid-abdomen displacing the spleen and stomach posterosuperiorly, the pancreas anteriorly and the left kidney inferiorly (fig. ). The left adrenal gland was not visible. A contrast-enhanced CT scan of the abdomen to further characterize the mass showed no infiltration of the surrounding vasculature or organs, and the left adrenal gland was visualized although it could not be confirmed if the mass was arising from the adrenals (fig. ). The suggested radiological differential diagnoses were: liposarcoma, teratoma and myelolipoma. A needle biopsy of this mass showed adipose tissue with foci of extramedullary hematopoiesis consistent with myelolipoma without any evidence of malignancy, confirming this mass to be a benign myelolipoma (fig. ).\nUrinary epinephrine, metanephrine and vanillylmandelic acid were within normal limits. A renal perfusion scan showed a small left kidney with reduced perfusion contributing 22% to the overall tubular function without obstructive uropathy. The lower extremity edema was partly ascribed to be due to inferior vena cava compression, and diuretics were decreased due to worsening renal function. Given the bradycardia and severe congestive heart failure, an electrophysiologist was consulted for evaluation and need for pacemaker implantation. Bradycardia was ascribed to be partly due to the Cushing reflex secondary to elevated blood pressure, and as the patient had no symptoms secondary to a low heart rate, pacemaker implantation was not advised. Despite maximum doses of clonidine, isosorbide, hydralazine, losartan and lisinopril, at best a moderate blood pressure control was achieved preoperatively.\nThe patient was taken for surgical removal of the mass. During surgery, the mass was found to be soft, highly vascular but well encapsulated and attached only to the left adrenal gland via a small portion that was resected along with the mass (fig. ). There was minimal blood loss during the surgery, and the patient had an uneventful recovery in the postoperative period. A renal perfusion scan done 8 days after surgical removal showed 36% contribution to the overall tubular function. Blood pressure control improved after surgery and the patient was gradually titrated off clonidine, hydralazine and lisinopril. Bradycardia resolved with few episodes of atrial fibrillation with rapid ventricular response that were controlled with β-blockers and digoxin. The antihypertensive medications that the patient was discharged on were carvedilol 40 mg, valsartan 320 mg and isosorbide mononitrate 240 mg.