MALIGNANT HYPERTENSION SECONDARY TO PHEOC HROMOCYTOMA IN A MAN WITH VONRECKLINGHAUSENS DISEASE

We present the case of a 29-year-old man with Von Recklinghausen's dis ease who developed malignant hypertension. The patient was admitted to our hospital because of visual disturbances caused by bilateral papil ledema. Physical examination revealed a blood pressure of 210/140 mmHg . Abdominal echography showed a large solid, hyperechoic suprarenal ma ss oh the rihgt side. An abdominal CT scan also revealed a right supra renal mass measuring about 6,5 cm diameter. Vanilmandelic acid and uri nary and plasma cathecholamines were 6 to 100-times their reference va lues. Scintigram with I-131-meta-iodo-benzylguanidine showed intense c oncentration of radioactivity in the right suprarenal gland without an y extra-adrenal abnormal deposit. Prior to a right adrenalectomy, phar macologic blockade and volume repletion were performed. A 6 cm diamete r mass was excised; its histologic diagnosis was consistent with pheoc hromocytoma. After this, blood pressure became normal and the symptoma tology dissappeared. 18 months later, the patient remains asymptomatic , normotensive without drugs and with normal serial results for catech olamines and their metabolites.