Intraneural perineurioma of the third cranial nerve: occurrence and identification. Case report

J Neurosurg. 2006 May;104(5):824-7. doi: 10.3171/jns.2006.104.5.824.


Intraneural perineurioma is a true but rare neoplasm that originates from perineurial cells and mainly affects peripheral nerves. It must be distinguished from other hypertrophic neuropathies that are either inflammatory or demonstrate an onion-bulb formation that originates from Schwann cells. Complying with this strict definition, only three additional cases of cranium-related perineurioma have been identified: two lesions arose extracranially and involved cranial nerves, and one occurred intracranially but did not involve a nerve. The authors describe a 27-year-old woman who presented with left third cranial nerve palsy and was found to harbor a mass lesion in the superior orbital fissure and cavernous sinus. After subtotal resection had been performed, pathological studies confirmed the presence of perineurial tumor cells in a pseudo-onion bulb formation. The cells stained positively for epithelial membrane antigen but not for S100 protein, clearly distinguishing the disease from one that originates in Schwann cells.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cavernous Sinus / pathology
  • Cavernous Sinus / surgery*
  • Cranial Nerve Neoplasms / diagnosis
  • Cranial Nerve Neoplasms / pathology
  • Cranial Nerve Neoplasms / surgery*
  • Diagnosis, Differential
  • Female
  • Humans
  • Image Processing, Computer-Assisted
  • Magnetic Resonance Imaging
  • Nerve Sheath Neoplasms / diagnosis
  • Nerve Sheath Neoplasms / pathology
  • Nerve Sheath Neoplasms / surgery*
  • Oculomotor Nerve Diseases / diagnosis
  • Oculomotor Nerve Diseases / pathology
  • Oculomotor Nerve Diseases / surgery*
  • Orbital Neoplasms / diagnosis
  • Orbital Neoplasms / pathology
  • Orbital Neoplasms / surgery*
  • Peripheral Nerves / pathology
  • Schwann Cells / pathology