Prevalence of C‐cell hyperplasia and medullary thyroid carcinoma in a consecutive series of pheochromocytoma patients

Abstract

Abstract During a 27‐year period (1956–1982), 64 patients were treated surgically for pheochromocytoma. There was no kinship among any of these subjects. The true prevalence of C‐cell disease of the thyroid in this series was searched for by performing serum calcitonin determinations after provocation with pentagastrin. Postoperative follow‐up of the pheochromocytoma disease was performed by blood pressure measurement and determination of urinary excretion of catecholamines and metabolites. Ten individuals (15.6%) were found to have C‐cell disease indicating that they had multiple endocrine neoplasia type II (MEN II) syndromes. Seven of these patients had been diagnosed before the time of follow‐up evaluation, 4 because of symptoms and 3 because of the screening procedure. At the systematic follow‐up, 3 “new” cases of C‐cell disease were diagnosed. As a result of the screening procedure, we found 4 “new” MEN II kindreds. After unilateral adrenalectomy, 7 of the 10 MEN II patients have been followed for more than 7.7 years without signs of recurrent adrenal disease. Our results suggest that the prevalence of MEN II syndromes among pheochromocytoma patients is higher than previously reported. Removal of only grossly evident pheochromocytomas and careful follow‐up is a good alternative to routine bilateral adrenalectomy in MEN II patients with pheochromocytoma.