Approach to the Patient With Primary Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1.

Area of Science:

• Endocrinology
• Genetics
• Surgical Oncology

Background:

• Multiple endocrine neoplasia type 1 (MEN1) is a rare genetic disorder causing multiple endocrine tumors.
• Primary hyperparathyroidism (PHPT) is the most frequent and earliest manifestation in MEN1 patients.
• Surgical intervention for PHPT in MEN1 is indicated for hypercalcemia, renal, or skeletal complications, ideally before age 50.

Purpose of the Study:

• To outline treatment goals for PHPT in MEN1, focusing on correcting hypercalcemia and preventing complications.
• To review current surgical strategies, including subtotal parathyroidectomy and individualized unilateral resection.
• To address the challenges of recurrent hypercalcemia and the management of ectopic parathyroid glands in MEN1.

Main Methods:

• Review of current literature on MEN1 and PHPT management.
• Analysis of surgical approaches for parathyroidectomy in MEN1 patients.
• Discussion of diagnostic and therapeutic strategies for recurrent disease and ectopic glands.

Main Results:

• Subtotal parathyroidectomy is the standard surgical approach, while individualized unilateral resection is an option for localized lesions.
• Recurrent hypercalcemia post-surgery is common in MEN1, often requiring reoperation or medical management.
• Ectopic parathyroid glands present significant localization and surgical challenges in MEN1-related PHPT, contributing to recurrence.

Conclusions:

• Timely surgical management of PHPT in MEN1 is crucial for preventing target organ damage.
• Managing recurrent hypercalcemia and ectopic parathyroid glands in MEN1 remains a significant clinical challenge.
• Advances in imaging and surgical techniques are needed to improve outcomes for MEN1 patients with PHPT.