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Thursday, 13 October 2011

Multiple endocrine neoplasia

- autosomal dominant
- MEN-1 gene is a tumor suppressor gene producing menin (chromosome 11)
- MEN-2 gene is ret proto-oncogen (receptor tyrosine kinase)

MEN-1 (Wermer's syndrome)      "PPP" 
- Parathyroid hyperplasia/adenoma
- Pancreas endocrine tumor (gastrinoma, insulinoma, somatostatinoma, VIPoma, glucagonoma)
- Pituitary prolactinoma or GH secreting tumor

MEN-2A (Sipple syndrome)          "TAP"
- Thyroid (medullary thyroid carcinoma)
- Adrenal (phaeochromocytoma)
- Parathyroid hyperplasia

MEN-2B
- similar to MEN-2A, but parathyroid hyperplasia is rare
- plus mucosal neuroma and Marfanoid appearance

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