The aim of this study was to compare 64 genetically determined pheochromocy
tomas (PPI) (49 MEN IIa, 3 MEN IIb, 6 Von Recklinghausen diseases, I von Hi
ppel-Lindau disease 5 familial pheochromocytomas) and 48 sporadic PH. Genet
ically determined PW were more often observed among men ann. more frequentl
y bilateral and multicentric than sporadic PH. Sporadic tumors had more oft
en adrenal capsular invasion necrosis and pseudocysts. Generically determin
ed PH were more differentiated with an insular pattern, hyaline globules an
d a higher percentage of polyedric cells. Sporadic tumors were less differe
ntiated with more frequently a diffuse pattern and small cells. Adrenal med
ullar hyperplasia was significantly associated with genetically determined
PH. Adrenal cortical hyperplasia was not associated with a particular type
of PH. The PS100 and chromogranin immunodetection was equivalent in both gr
oups.