Multiple endocrine neoplasia
From IDWiki
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
