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Malignant Spinal Cord Compression

  1. Usual primaries
    1. Lung
    2. Breast
    3. Prostate
    4. Lymphoma
  2. MC and first symptoms — Back pain
  3. MC segment affected — Thoracic cord
  1. Neurocutaneous Syndromes Q
    2. AD inheritance
  2. Neurofibromatosis I: (Von Recklinghausen’s Disease)
    1. Characteristic
      1. Cutaneous neurofibromas
      2. Café ‘au’ lait spots
      3. Freckling in non sun exposed areas (eg. axilla)
      4. Lisch nodules (Iris hamartomas)
      5. Pseudoarthrosis of tibia
    2. Mutation of NFl gene (tumor suppressor gene) encoding ‘NEUROFIBROMIN’ on Ch 17 causes the syndrome.
    3. Associated with
      1. Neuroma
      2. Schwannoma
      3. Meningioma
      4. Optic glioma
      5. Ependymomas
      6. Astrocytoma
      7. Pheochromocytoma
    4. Neurofibroma
      Occurs in subcutaneous tissue, slightly painful, firm, smooth, movable in lateral direction of nerve, associated with paraesthesia & tingling. Cystic degeneration & sarcomatous changes are rare. Neurofibromas may also grow from sheath of a peripheral nerve or a cranial nerve. They are difficult to remove without removal of nerve itself.
  3. Neurofibromatosis 2:
    1. Café-au-lait spots, peripheral neurofibromas occur rarely.
    2. Predisposition for
      1. Meningioma - Gliomas
      2. Schwanomas of cranial & spinal neves
    3. Mutation of NF2 gene encoding MERLIN (Ch. 22)
  4. Tuberous Sclerosis (Bourneville’s Disease)
    1. Characterized by —
    2. Cutaneous lesions:
      1. Adenoma sebaceum (facial angiofibromas)
      2. Ash leaf shaped hypopigmented macules (best seen under ultraviolet illumination with a Wood’s lamp)
      3. Hagreen patches (yellowish thickenings of skin over lumbosacral region of back)
      4. Depigmented nevi.
    3. Subependymal nodules on ñeuroimaging studies are characteristic.
      1. Mutation is ISC 1 & ISC 2 (seconding for HAMARTIN & TUBERIN).
      2. Predisposition for ependymomas and childhood astrocytoma.
    4. Other associations:
      1. Rhabdomyomas of myocardium
      2. Angiomyomas of kidney, liver, adrenals and pancreas
    5. Treatment: Q
      1. Symptomatic
      2. Anticonvulsants for seizures, shunting for hydrocephalus and behavioral and educational strategies for mental retardation are mainstays of management.
    Characterized by —
    1. facial nerve flammens
    2. C/L focal seizures
    3. Calcification of cortex/subcortical structures
    4. I/L glaucoma
    1. Retinal and cerebellar hamangioblastomas
    2. Spinal cord angiomas, cystic tumors of pancreas, kidney and epididymis
    3. Associated with nystagmus, ataxia and high ICP.
    1. AR, Ch.11 involved
    2. Ass. with progressive cerebellarataxia, oculocutaneous telangiectoxia, choreoathetosis, pulmonary and sinus infections, lymphoreticular malignancy.

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