Intradural extramedullary spine tumors

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Initial posting from: Bernard Bendok, MD

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1. Neurofibroma

  • No sex predilection, evenly distributed throughout spine, 20% extradural
  • 70-80% present with local or radicular pain, 40-60% present with motor dysfunction, 20-30% present with bladder dysfunction
  • From many cell types, not all of which may be transformed Schwann cells, perineural cells, fibroblasts, lymphocytes, mast cells
  • Associated with mutation in NF1 gene (Neurofibromin) and Neurofibromatosis Type I
  • Arise WITHIN a nerve, producing a fusiform mass that incorporates the nerve

2. Schwannoma

  • No sex predilection, evenly distributed throughout spine, 30% extradural
  • 70-80% present with local or radicular pain, 40-60% present with motor dysfunction, 20-30% present with bladder dysfunction
  • From neural-crest derived Schwann cells (S-100 and leu+)
  • Even sporadic cases associated with NF2 gene mutation (merlin) or absence of its gene product; also associated with neurofibromatosis type II.
  • Encapsulated, globular mass that growns ON a nerve, DISPLACING it to the side, rarely entrapping it
  • Antoni A (high cellularity) and B (less densely cellular) areas and Verocay Bodies

3. Meningioma

  • 4:1 female predilection, 80% in T spine, 10% extradural
  • 40-70% present with locla or radicular pain, 80% present with motor dysfunction, 50% present with bladder dysfunction
  • May cause Brown Sequard
  • Derived from arachnoid cap cells
  • Usually missing part of 22q hence the association with NF2
  • Often progesterone receptor positive
  • Associated with radiation exposure
  • Cellular whorls, psammoma bodies (often many in spinal meningiomas)

4. Epidermoid Cyst

  • Often lateral to midline
  • Hypointense on T1, Hyperintense on T2
  • Acquired secondary to trauma (lumbar punctures)

5. Dermoid Cyst

  • Often midline
  • Often at thoracolumbar junction, conus medullaris and cauda equina
  • Hyperintense on T1, Hypointense on T2
  • Can rupture, leading to a chemical meningitis

6. Sarcoma

7. Metastatic Disease

Bernstein, M and Berger, M ed. Neuro-Oncology. New York: Thieme Medical Publishers, Inc, 2000.

Kumar, V, Fausto, N, Abbas, A ed. Robbins & Cotran Pathologic Basis of Disease, 7th Ed. Philadelphia: WB Saunders Company, 2004.

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