Oncogenes/tumor suppressor genes

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  1. Tumor suppressor genes
    1. astrocytomas
      1. DNA is commonly lost on chromosomes 10p, 17p, 13q, and 9
    2. oligodendrogliomas
      1. frequently have deletions of 1p and 19q
    3. meningiomas
      1. portions of 22q are often lost (also contains gene for NF2)
  2. Oncogenes
    1. astrocytomas
      1. malignant gliomas usually develop from one of two routes
        1. slower growing with deletions of chromosome 17 and inactivation of the p53 gene
        2. tumor presenting more malignant with amplification of epidermal growth factor receptor (EGFR) and an intact p53
        3. in both cases there is often a loss of chromosome 10
    2. hereditary syndromes associated with brain tumors
      1. NF1 – 17q coding for neurofibromin resulting in neuromas, schwannomas, meningiomas, and optic gliomas
      2. NF2 – 22p coding for merlin leading to schwannomas, gliomas, ependymomas, meningiomas
      3. Tuberous sclerosis – 9q or 16p coding for hamartin and associated with astrocytomas
      4. VHL – 3p coding for pVHL (modulator of mRNA elongation) resulting in hemangioblastomas of retina, cerebellum and spinal cord; pheochromocytomas
        1. Remember: 3 letters in VHL and VHL is on chromosome 3
      5. Li-Fraumeni – p53 (17p) resulting in malignant gliomas, choroids plexus papillomas
      6. Retinoblastoma – 13q causing retinoblastoma (radiation sensitive), pineoblastoma, and malignant glioma
      7. Turcot – 5q associated with medulloblastoma, malignant glioma
      8. MEN I – 11q coding for menin and associated with pituitary adenoma and malignant schwannoma
      9. Familial melanoma – 9p
      10. Familial breast cancer – 17q and 13q (BRCA1 and 2 respectively)
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