[Familial pheochromocytoma and Van Hippel-Lindau disease]

The authors report the case of a 41-year old man who presented simultaneously with phaeochromocytoma, paraganglioma and renal carcinoma. The postoperative finding of metaiodobenzylguanidine uptake foci showed that the phaeochromocytoma was malignant. The patient's son had a phaeochromocytoma and a pancreatic cyst. The phaeochromocytoma had no clinical manifestations and had been discovered by systematic assays of urinary catecholamines and their derivatives. These abnormalities are those of Von Hippel-Lindau disease.