Multiple Endocrine Neoplasia

 

  • MEN 1

    Aka Wermer's syndrome.

    Autosomal dominant mutation in MEN1 gene. Tumours in 3Ps (1 letter):

    • Parathyroid (PTH).
    • Pituitary: especially prolactinoma.
    • Pancreatic: gastrinoma (Zollinger-Ellison syndrome), insulinoma.
    • Also carcinoid tumours in GI tract, and skin lesions such as angiofibromas.

  • MEN 2

    Autosomal dominant mutation in RET oncogene.

    MEN 2a is 2P1M:

    • PTH
    • Phaeochromocytoma
    • Medullary thyroid cancer (MEN two).

    MEN 2b is 1P3M:

    • Phaeochromocytoma
    • Medullary thyroid (MEN two).
    • Marfanoid appearance.
    • Mucosal neuromas: yellow-white nodules on lips and tongue.

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