MEN Type I (Wermer Syndrome)

Area of Science:

• Endocrinology
• Oncology
• Genetics

Background:

• Multiple Endocrine Neoplasia type I (MEN I) is an autosomal dominant genetic disorder.
• It is characterized by hyperplastic or neoplastic changes in the parathyroid, pituitary, and endocrine pancreas.
• Hyperparathyroidism is the most frequent clinical manifestation of MEN I.

Observation:

• A 42-year-old female patient presented with symptoms of hyperparathyroidism and multiple bone pains.
• She was diagnosed with parathyroid adenoma, pancreatic neoplasm, and adrenal adenoma.
• Initial treatment focused on hyperparathyroidism via hemiparathyroidectomy.

Findings:

• Post-hemiparathyroidectomy, the patient experienced a significant decrease in serum calcium and parathyroid hormone (PTH) levels.
• Serum Ca and PTH levels normalized, leading to remarkable improvement in bone pain.
• Coincident pancreatic and adrenal neoplasms were noted but asymptomatic, thus managed conservatively initially.

Implications:

• This case underscores the importance of a multidisciplinary approach for managing MEN I.
• Effective surgical and medical management can significantly improve patient outcomes and quality of life.
• Early diagnosis and tailored treatment are crucial for addressing the diverse manifestations of MEN I.