Introduction: Pheochromocytoma is an uncommon tumor. Non-classical forms can make diagnosis difficult and delay correct management strategies.
Case report: A 20-years old caucasian male presented to the emergency room with unproductive cough, fever and tiredness for 7 days. He was eupneic, afebrile, BP 176/115 mmHg, 104 bpm with inspiratory crepitations in the left lung base. Leucocyte14.1×109/l (N 70%); platelets 659×109/l; CRP 6.9 mg/dl. Normal renal and hepatic function tests and no biochemical markers of myocardial lesion. X-ray: cardiomegaly, opacities in both lungs. Echocardiogram: dilated ventricles, LVH and LVEF 37%. Diagnosed as bilateral pneumonia, dilated myocardiopathy and hypertension. On day 4, acute right limb ischemia, hemodinamic instability, high fever and rapid deterioration of renal function. CT scan: 7 cm left adrenal tumor, aortic enlarged lymph nodes, right kidney hyperdensity suggesting embolism. Pheochromocytoma was confirmed by urinary epinephrine 202 μg/day (<27) norepinephrine 311 (<97), and 131MIBG hyperfixation. After phenoxibenzamine he underwent left adrenalectomy and loco-regional limphadenectomy. Three days later a left nephrectomy was necessary due to irreversible ischemia. Abdominal hematoma and accentuation of thrombocytosis supervene. Hystology: pheochromocytoma (unknown behaviour). One month later, he developed hypercalcemia 11.9 mg/dl, PTH <3 pg/ml, with normal PTHrp and chromogranin A. Thrombocytosis and hypercalcemia both subsided in the next 3 months. No gene mutations were found. After 30 months the patient is asymptomatic.
Conclusions: Dilated myocardiopathy with heart failure in a young hypertensive patient should lead to consider pheochromocytoma. Occlusive arterial phenomena and hypercalcemia made this case even more complex. Tumor size and histology justifies lifelong control.
30 Apr - 04 May 2011
European Society of Endocrinology