Endocrine Abstracts (2011) 26 P360

Pheochromocytoma: a complex case

F Fonseca1, A Cordero2, J Oliveira1, L Mourão1, J Coutinho1 & A Agapito1

1Curry Cabral Hospital, Lisboa, Portugal; 2Santa Luzia Hospital, Elvas, Portugal.

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. Leucocyte–14.1×109/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.

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