Multiple Endocrine Neoplasia (MEN)| | | | | | | | Type 1
(Wermer Syndrome) | | – AD, MEN1 mutation (menin: tumor suppressor)
– Angiofibromas, collagenomas, lipomas, CALMs
– Pituitary, parathyroid, pancreatic tumors | | | Type 2a
(Sipple Syndrome) | | – AD, RET mutation (tyrosine kinase receptor)
– Lichen or macular amyloidosis, hemangiomas, genital lentigines, hamartomas, lipomas
– Parathyroid tumor, thyroid medullary carcinoma, pheochromocytoma | | | Type 2B
(Multiple Mucosal Neuroma Syndrome) | | – AD, RET mutation
– Multiple mucosal neuromas, thickened lips
– Thyroid medullary carcinoma, pheochromocytoma, marfanoid habitus, diffuse ganglioneuromatosis (megacolon, diarrhea) |
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| | | | | | MEN 1: 3 P’s (pituitary, pancreas, parathyroid) + CALMs
MEN 2A: Amyloidosis (“sipple” syndrome: think “rippled” macular amyloid)
MEN2B: Blubbery lips due to mucosal neuromas | | | | | |
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