SPORADIC AND FAMILIAR FORMS OF PHEOCHROMO CYTOMA - COMPARATIVE CLINICAL ANALYSIS

We reviewed the medical records of patients with pheochromocytoma diag nosed in our institution, to confirm the high frequency of association between pheochromocytoma and neurofibromatosis that we had found in a previous study, and to compare the main clinical and pathological fea tures of sporadic tumors and those with familial occurrence. Fourteen patient (8 women and 6 men) with mean age 43.1 +/- 12.3 years were stu dies. They were separated into group I: sporadic tumors (n = 9) and gr oup II: tumors associated with familial syndromes (n = 6). The clinica l features, biochemical results (from measurements of 24-hour urinary vanilymandelic acid, catecholamines and metanephrines), and location a nd size of the tumor were compared between both groups. One patient ha d a malignant pheochromocytoma, another had bilateral tumors and a thi rd patient had an extra-adrenal pheochromocytoma. Neurofibromatosis oc curred in association with pheochromocytoma in 4 of 14 patients (28,5 %), and MEN II was diagnosed in another. We have found no differences between the two groups except time to diagnosis that was shorter in th e group with familial occurrence (6.4 +/- 9 vs 56.7 +/- 47 months; p = 0.039). The present study confirms the high frequency of pheochromocy toma associated with neurofibromatosis in our country. These data sugg est the need for pheochromocytoma screening in patient with neurofibro matosis and their families.