Mindmap - MEN$

Multiple Endocrine Neoplasia Syndrome.

Multiple Endocrine Neoplasia $

• general characteristics
  • group of inherited diseases
    in Multiple endocrine organs
    resulting in proliferative lesions
    • hyperplasia
    • adenoma
    • carconoma
    certain distinctive features in
    contrast to sporadic neoplasia
    • younger age
    • multiple endocrine organs
    • synchronously / metachronously
    • preceded by an asymptomatic stage of hyperplasia
    • the tumours are usually more aggressive
    • recur in a higer proportion of cases
• MEN type 1
  • autosomal dominant pattern
  • gene location at 11q13 - tumour suppressor gene
    • inactication of both alleles
      believed to be basis of tumorigenesis
  • organs involved -
    the 3 P's
    • Parathyroid (95%)
      • primary hyperparathyrodism, arising from multiglandular hyperplasia
    • Pancrease (40%)
      • leading cause of death in MEN1
      • usually aggresive and present with metastatic disease
      • often functional.
        • Zollinger-Ellison $
          • triad of 
            gastric acid hypersecretion, 
            severe peptic ulceration,
            non-beta cell islet tumor of pancreas (gastrinoma).
        • Hypoglycemia
          • insulinoma
    • Pituitary (30%)
      • prolactin-secreting macroadenoma
      • somatotropin-secreting tumours
        • acromegaly
• MEN type 2
  • unified by the occurance of activation of RET protooncogene
  • autosomal dominant
  • 2A
    • Thyroid
      • medullary carcinoma of thyroid
        • multifocal
        • first 2 decades of life
        
    • Adrenal medulla
      • 50% develop adrenal pheochromocytomas
        • no more than 10% malignant
        
    • Parathyroid
      • 1/3 develop Parathyroid gland Hyperplasia with primary Hyper-PTH 
  • 2B
    • unlike 2A
      • no primary hyperparathyrodism
      • develop extraendocrine manifestations
        • ganglioneuromas of mucosa
          • GI tract
          • lips
          • tongue
        • marfanoid habitus
    • organs affected
      • Thyroid
      • Adrenal medulla

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