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Chapter: Pathology: Endocrine Pathology

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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.

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, pheo-chromocytoma, 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

 

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