Multiple endocrine neoplasia type 1 parathyroid adenoma development over time

Abstract

Multiple gland parathyroid disease is one of the hallmarks of multiple endocrine neoplasia (MEN) type 1. Often mislabeled parathyroid hyperplasia, the process is actually the development of multiple adenomas. Some clinicians have reported results of selective parathyroidectomy in this group, removing only grossly enlarged glands. We argue that all the glands are at risk and should be addressed at any planned parathyroid intervention. Our hypothesis is that, given sufficient time, patients would all develop adenomas in each of the parathyroid glands. Our available data to address this issue are the parathyroidectomy results from a single institution series. Patients who had initial parathyroid exploration for hyperparathyroidism in the setting of MEN-1 were reviewed. This study includes those patients who had the weights of the resected glands documented; 23 men and 21 women met the criteria. The total weight of the parathyroid glands did not vary with the age of the patient at operation. However, the number of normal glands identified did vary significantly with age (p < 0.02), with older patients being less likely to have any normal parathyroid glands. Although total parathyroid weight may correlate with development of hypercalcemia and indications for operation, the involvement of multiple parathyroid glands in MEN-1 is a function of time, as independent events in each gland must occur. Given time, MEN-1 patients all develop multiple gland disease, and this reality must be used in planning operative management for patients with this syndrome.