Intraoperative Decision-Making and Technical Aspects of Parathyroidectomy in Young Patients With MEN1 Related Hyperparathyroidism

Abstract

One in 5,000 to 1 in 50,000 births have multiple endocrine neoplasia type 1 (MEN1). MEN1 is a hereditary syndrome clinically defined by the presence of two of the following endocrine tumors in the same patient: parathyroid adenomas, entero-pancreatic endocrine tumors and pituitary tumors. Most commonly, patients with MEN1 manifest primarily with signs and symptoms linked to primary hyperparathyroidism. By age 50, it is estimated that 100% of patients with MEN1 will have been diagnosed with primary hyperparathyroidism. These patients will need to undergo resection of their hyperfunctioning glands, however there is no clear consensus on which procedure to perform and when to perform it in these patients. In this original study we describe and explain the rational of our peri-operative approach and management at MD Anderson Cancer Center of MEN1 patients with hyperparathyroidism. This protocol includes preoperative evaluation, intraoperative decision-making and detailed surgical technique adopted for these patients' care. Additionally we review follow-up and disease management in instances of recurrent primary hyperparathyroidism in patients with MEN1 syndrome.

Multiple Endocrine Neoplasia Type 1 (MEN1): An Update and the Significance of Early Genetic and Clinical Diagnosis

“…However, some groups perform pre-operative imaging in order to evaluate for ectopic parathyroid glands, or more recently, in order to plan for less extensive surgeries, but these protocols need to be validated by large series and post-operative long-term follow-up (66–69). Bilateral neck exploration during surgery should be planned in an attempt to identify all pathological parathyroid glands (70).…”

Video-assisted thoracoscopic surgery for primary hyperparathyroidism with ectopic parathyroid adenoma in thymus

“…Inferior parathyroid glands and thymus both originate from the third pharyngeal arch [3,6,13]. Other common localizations of ectopic parathyroid tissue include mediastinum, thyroid gland, and parapharyngeal space [3,9,[14][15][16][17]. Signs and symptoms of hyperparathyroidism can also be the first presentation of multiple endocrine neoplasia type 1 (MEN1).…”

It's not a mystery, it's in the history: Multidisciplinary management of multiple endocrine neoplasia type 1

“…In MEN1, the initial surgical intervention of choice is subtotal parathyroidectomy. 24 Total parathyroidectomy with autotransplantation of parathyroid tissue to the forearm as an initial operation risks the devastating sequelae of hypoparathyroidism. 25 Concomitant transcervical thymectomy is suggested at the initial operation to decrease the rate of recurrence, as supernumerary glands in the thymus can occur, and theoretically lessen the risk of thymic NET development.…”

“…In 2011, the low bone mass for age prompted the decision for surgery. In MEN1, the initial surgical intervention of choice is subtotal parathyroidectomy 24 . Total parathyroidectomy with autotransplantation of parathyroid tissue to the forearm as an initial operation risks the devastating sequelae of hypoparathyroidism 25 .…”

“…He had facial angiofibromas and lipomas. Laboratory testing revealed an inappropriately normal intact parathyroid hormone (iPTH) level of 44 pg/mL (normal range, 15-65 pg/ mL), a normal 25-hydroxy vitamin D3 level of 33 ng/ mL (normal range, 25-80 ng/mL), and high-normal 24hour urinary calcium of level 237 mg (normal range, 25-300 mg), which was concerning for primary hyperparathyroidism (PHPT). He reported a family history of nephrolithiasis, constipation, and metastatic thymic neuroendocrine tumor (NET) in his father and pituitary macroadenoma in his paternal uncle.…”