Multiple endocrine neoplasia

Definition

• Genetic disorder predisposing to the development of endocrine tumours

Classification

• MEN-1 (Wermer syndrome) ("PPP")
  • Parathyroid (hyperparathyroidism)
  • Pancreas (gastrinomas, insulinomas)
  • Pituitary (prolactinoma, growth-hormone-producing adenomas
• MEN-2A ("MPP")
  • Medullary thyroid carcinoma (almost all patients)
  • Pheochromocytoma (40-50%)
  • Parathyroid hyperplasia (10-20%)
• MEN-2B ("MMP")
  • Medullary thyroid carcinoma
  • Marfanoid habitus
  • Pheochromocytomas (often bilateral)

Management

MEN-1

• Often presents with primary hyperparathyroidism
• Gastrinomas may present with Zollinger-Ellison syndrome
• Insulinomas may present with hypoglycemia
• Signs of hyperprolactinemia
• Signs of acromegaly