Advances in the diagnosis and management of primary hyperparathyroidism due to MEN type 1 syndrome

Multiple endocrine neoplasia type 1 (MEN1) was first described in 1954. Since then, we have discovered over 1100 germline mutations associated with it, and it affects 1 in 30, 000 births worldwide. It is estimated that by age 50, 100% of patients with MEN1 will have primary hyperparathyroidism (PHPT). These patients need to undergo a parathyroidectomy with the objective to attain long-term normocalcemia, avoid postoperative hypoparathyroidism, and facilitate reexploration in cases of persistent or recurrent PHPT. However, there is no clear consensus on which technique is most appropriate to achieve these goals. Through this review, we aim to discuss the three main procedures associated with treating PHPT in MEN1 patients: less than subtotal parathyroidectomy, subtotal parathyroidectomy, and total parathyroidectomy with autotransplantation. Additionally, we reviewed preoperative workup, follow-up, and recurrence management in these patients. A brief description of the authors’ surgical technique with intraoperative decision-making has been included.