Management of primary hyperparathyroidism in MEN1: From initial subtotal surgery to complex treatment of the remaining gland

Abstract

Multiple endocrine neoplasia type 1 (MEN1) is a rare genetic disease with autosomal dominant transmission, which can cause various tumors, particularly endocrine, in a given patient. Primary hyperparathyroidism (PHPT) is the most common and earliest manifestation, leading to surgery before the age of 50 in most patients. Biological severity and renal and/or bone complications dictate the timing of parathyroid surgery. The objective is to correct hypercalcemia to prevent impact, while minimizing the risk of hypoparathyroidism. The most widely recommended procedure is subtotal parathyroidectomy (3 or 3.5 glands removed), with thymic horn resection via a cervical route. The development of imaging techniques, however, makes it possible to discuss partial surgery (resection of 1 or 2 glands) on a case-by-case basis depending on preoperative imaging and other elements such as patient age. Finally, hypercalcemia recurrence after initial surgery is a common feature of MEN1, and management of the remaining gland is challenging with various options: reoperation, calcimimetics and US-guided ablation or therapeutic abstention.