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November 1996

Sporadic Primary Hyperparathyroidism in the Setting of Multiple Endocrine Neoplasia Type 1

Author Affiliations

From the Department of Molecular Medicine, Karolinska Hospital, Sweden (Drs Teh and Larsson); and Royal Hobart Hospital, Tasmania, Australia (Drs McArdle, Parameswaran, and Shepherd and Mr David).

Arch Surg. 1996;131(11):1230-1232. doi:10.1001/archsurg.1996.01430230112020

Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant disease characterized by neoplasia involving the parathyroid glands, the endocrine pancreas, the duodenum, and the anterior pituitary. The most commonly involved gland is the parathyroid gland, which has been found to be abnormal in 90% to 95% of all patients with MEN 1. The disease process is invariably multiglandular and histologically described as either hyperplasia or multiple adenoma, although the histological distinction 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 adenoma is estimated to be as high as 1 in 100.2,3 We report herein the first case of sporadic primary hyperparathyroidism in the setting of MEN 1 based on clinical, biochemical, pathological, and genetic studies.

REPORT OF A CASE  In 1986, we examined a 53-year-old woman

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