No Country for Old ________........

(Does this man have acromegaly?)











Multiple Endocrine Neoplasia (MEN) syndromes can often be a bit confusing. It should be considered in those with....uhhhh....endocrine neoplasia - at multiple sites.

MEN 1: An autosomal dominant disease with mutations in the MENIN gene.
Remember "the 3 P's":

• Pituitary tumors: particularly the anterior pituitary.
• Pancreas: islet cell tumors like Insulinomas, Glucagonomas, and VIPomas.
• Parathyroid hyperplasia, usually in all glands.

MEN 2: Also an autosomal dominant disease. The mutation is in the RET proto-onco gene.

• MEN 2A: aka Sipple Syndrome
  
• Medullary thyroid cancer; typically bilateral. In non-MEN disease this is mostly unilateral.
  
• Pheochromocytoma in about 50%. Also can be bilateral.
• Parathyroid hyperplasia

• Men 2B
• Medullary thyroid cancer: as above.
• Parathyroid hyperplasia: as above.
• Marfanoid habitus: high arched palate, pectus excivatum.