MEN syndromes

Inherited as autosomal dominant

All family members of a patient diagnosed with MEN syndromes should be screened for MEN.

Types:  MEN I, MEN IIA, MEN IIB

MEN I

Most common tumors:  mnemonic – PPP (all the P’s are followed by a vowel)

• Parathyroid hyperplasia (90% of patients have this)
• Pancreatic islet cell tumors (66%):  Gastrinoma (ZE syndrome) 50%, Insulinoma 20%
• Pituitary tumors (66%)

Other tumors associated with MEN I = adenomas of Adrenal 30% and Thyroid 15%

MEN IIA

Most common tumors: mnemonic MPH – 2 miles per hour

• Medullary thyroid carcinoma 100%
• Pheochromocytoma 33%
• Hyperparathyroidism (50%), hypercalcemia

100% of patients with MEN IIA have medullary carcinoma of the thyroid.

MEN IIB

Most common abnormalities: mnemonic MMMP – 3M plastics

• Mucosal neuromas 100%
• Medullary thyroid carcinoma 85%
• Marfanoid
• Pheochromocytoma 50%

Physical findings of MEN IIB:  Mucosal neuromas (mouth, eyes, etc), Marfanoid body shape, abnormal arch of foot, constipation.

Major difference between MEN IIA and IIB:  MEN IIA has Hyperparathyroidism – hyperplasia, MEN IIB does not.

MEN I and MEN IIA have hyperplasia of the parathyroid – treated with removal of all parathyroid tissue with autotransplant of some parathyroid tissue – perhaps into the forearm.

Picture credit:

http://commons.wikimedia.org/wiki/User:Mikael_H%C3%A4ggstr%C3%B6m