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Nineteen of 82 unselected patients with pheochromocytomas(23 percent)were carriers of familial disorders;19 percent had von Hippel-Lindau disease and 4 percent had MEN-2.Prospectively,in 36 of 79 subjects at risk for pheochromocytoma(46 percent),42 unsuspected pheochromocytomas were found. Overall,there were 130 patients with 185 pheochromocytomas;43 had von Hippel-Lindau disease,24 had MEN-2,and 63 had sporadic tumors.The patients with familial and those with sporadic pheochromocytomas differed in mean age at diagnosis(32 vs.46 years,P<0.001),multifocal localization(55 vs.8 percent,P<0.001)and cancer(0 vs.11 percent,P=0.005);but not in the frequency of extraadrenal tumors(24 vs.16 percent).Thirty-eight percent of carriers of MEN-2 had pheochromocytoma as the only manifestation of their syndrome.All patients with pheochromocytomas should be screened for MEN-2 and von Hippel-Lindau disease to avert further morbidity and mortality in the patients and their families.All patients in families with MEN-2 or von Hippel-Lindau disease should be screened for pheochromocytoma,even if they are asymptomatic. |
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