[Surgical treatment of primary hyperparathyroidism during MEN1 and other genetically determined forms]

In MEN1, HPT manifests precociously and it is almost constant. Whereas in MEN2 it seldom occurs and has been reported in 17 to 40% of the cases, in MEN1 HPT is much more severe, amplifying the secretion of associated gastrinomas. In MEN1, multiglandular involvement is almost constant, and in case of less than subtotal parathyroidectomy, recurrence rate varies from 20 to 40%. Practically, HPT is the first worry of the surgeon in a MEN1 setting, and the last in a MEN2 setting. It is rather important to think to the potential hypoparathyrodism at the time of the cervicotomy for MTC. The operative strategy is to examine all parathyroid glands. In MEN1, it is necessary to be aggressive and perform a subtotal parathyroidectomy, regardless of gland gross appearance. In the MEN2 only the enlarged glands should be removed. In both setting a bilateral cervical thymectomy should be done for removal of a possible supernumerary gland.