Multiple Endocrine Neoplasia Syndromes

2021-02-21 12:00 AM

Multiple endocrine neoplasia (MEN) syndromes are autosomal dominant conditions with incomplete penetrance that are characterized by hyperplasia and tumours of endocrine glands occurring at a young age.

MULTIPLE ENDOCRINE NEOPLASIA SYNDROMES

Multiple endocrine neoplasia (MEN) syndromes are autosomal dominant conditions with incomplete penetrance that are characterized by hyperplasia and tumors of endocrine glands occurring at a young age.

  • MEN 1 (Werner syndrome) features tumors of the pituitary gland, parathy-roids, and pancreas.
  • Associated with peptic ulcers and Zollinger-Ellison syndrome

Affected gene is MEN1, a tumor suppressor gene which encodes a nuclear protein called menin

  • MEN 2A (Sipple syndrome) features medullary carcinoma of the thyroid, pheochromocytoma, and parathyroid hyperplasia or adenoma.

Mutation of RET proto-oncogene

  • MEN 2B features medullary carcinoma of the thyroid, pheochromocytoma, and mucocutaneous neuromas.
  • Mutation of RET proto-oncogene