EXTRAADRENAL AND MULTIPLE PHEOCHROMOCYTOMAS - ARE THERE REALLY ANY DIFFERENCES IN PATHOPHYSIOLOGY AND OUTCOME

In 15-20% of the cases pheochromocytoma (pheo) localizes in extraadren al sites and in about 15% of all cases it seems to be multiple. We ana lyze our 20-year experience in surgical treatment of pheos, studying t he differences between typical and extraadrenal or multiple tumors. Fr om 1977 to 1996 we operated 55 patients (patients) with pheos, 28 (50. 9%) males and 27 females (mean age 41 years, range 10-63). Two groups have been distinguished: classic pheos (Group 1, 45 patients) and extr aadrenal or multiple pheos (Group 2, 10 patients). Hypertensive crises were present in 37/45 (82.2%) patients of Group I and in 7/10 patient s of Group 2. Five (11.1%) masses were nonfunctioning and incidentally discovered (4 in Group 1 and one in Group 2). In 4 cases association with familial syndromes was observed (3 MEN IIb, 1 von Recklinghausen syndrome); no further significant differences in clinical features and laboratory data were found between the two Groups. At immunohystochem ical analysis 26/26 patients resulted positive for chromogranin A and NSE and 17/26 (11/20 in Group 1 and 4/6 in Group 2) resulted positive for S 100 protein. Five (11.1%) malignant pheos were discovered and re moved (Group 1); average survival of these patients was 54.4 months, t wo patients underwent radioactive iodine (131-I-MIBG) therapy after su rgery and only one patient is still alive at 24-month follow-up. Recur rence for benign sporadic pheo (Group 1) occurred in one patient 183 m onths after adrenalectomy. Ectopic, associated with familial syndromes and multiple pheos are not uncommon and although recovery in surgical ly treated patients is excellent, lifelong follow-up is necessary also in benign tumors.