SPORADIC PRIMARY HYPERPARATHYROIDISM IN THE SETTING OF MULTIPLE ENDOCRINE NEOPLASIA TYPE-1

Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant d isease characterized by neoplasia involving the parathyroid glands, th e endocrine pancreas, the duodenum, and the anterior pituitary, The mo st commonly involved gland is the parathyroid gland, which has been fo und to be abnormal in 90% to 95% of all patients with MEN 1. The disea se process is invariably multiglandular and histologically described a s either hyperplasia or multiple adenoma, although the histological di stinction between the 2 entities remains difficult and controversial.( 1) The prevalence of MEN 1 is approximately 0.002 in 100, whereas the prevalence of sporadic primary hyperparathyroidism due to a solitary a denoma is estimated to be as high as 1 in 100.(2,3) We report herein t he first case of sporadic primary hyperparathyroidism in the setting o f MEN 1 based on clinical, biochemical, pathological, and genetic stud ies.