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Updated: Oct 13 2017

Multiple Endocrine Neoplasias

Snap Shot
  • A 36-year-old women complains of severe episodes of headache, tremulousness, palpitations, and anxiety. The patient has noted a change in her voice and she has difficulty swallowing solids. On PE there is a palpable, nontender swelling in the front of her neck that moves with degluttition. There is not cervical lymphadenopathy. Lab studies show hypercalcemia. An X-ray of the cervical region shows irregular calcifications in mass, while an MRI of the abdomen shows confirms the presence of bilateral adrenal lesions.
    (Type IIA)
Introduction

  • Cancer syndrome with several associated endocrine neoplasms
    • concurrance occurs secondary to each organ's common embryologic origin
  • All MEN syndromes have AD inheritance 
  • There are 3 types
    • MEN I (Wermer's syndrome)
      • parathyroid tumors
      • pituitary tumors (prolactin or GH)
      • pancreatic islet cell/endocrine tumors
        • Zollinger-Ellison syndrome
        • insulinomas
        • VIPomas 
          • present with WDHA syndrome
            • Watery Diarrhea
            • Hypokalemia
            • Achlorhydria
        • glucagonomas
      • presentation
        • gastric ulcers
          • secondary to gastrin production by ZE syndrome
        • kindey stones
          • secondary to increased serum calcium as result of increased PTH
    • MEN IIA (Sipple's syndrome) 
      • medullary thyroid carcinoma  
        • secretes calcitonin
      • pheochromocytoma
      • parathyroid tumors
    • MEN IIB
      • medullary thyroid carcinoma
      • pheochromocytoma
      • oral/intestinal ganglioneuromatosis
        • associated with marfanoid habitus 
  • MEN IIa and IIb share several common features (reason it is not MEN II and III)
    • medulllary thyroid carcinoma
    • pheochromocytoma
    • ret association
 
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