Bilateral adrenal gland pheochromocytomas in the course of von Hippel-Lindau's disease

The pheochromocytoma is a medullo-adrenal tumor which develops at the cost of the chromaffin cells. It appears in 11-19% of cases of von Hippel-Lindau 's disease (VHL), is often bilateral, and the symptomatology is often crude : arterial hypertension is frequently isolated and unstable, and the classi c triad of headache, palpitations and sweating is quite rarely observed. We report four observations of bilateral pheochromocytomas in patients with v on Hippel-Lindau's disease (three with phenotype IIA and one with phenotype IIB). The tumor was bilateral during the diagnosis in three cases; in the fourth patient, the attack on the contralateral adrenal gland came two year s after the first adrenalectomy. All the patients had undergone an adrenale ctomy by open surgery after a short preparation of 48 hours; replacement th erapy was begun in each patient. Morbidity was low, and the patients submit ted to a prolonged follow-up in order to screen for the onset of future les ions of VHL. (C) 2001 Editions scientifiques et medicales Elsevier SAS.