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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