Intraneural perineurioma of the third cranial nerve: occurrence and identification

Case report

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  • 1 Semmes–Murphey Clinic and Department of Neurosurgery, University of Tennessee Health Science Center; and Pathology Group of the MidSouth, Memphis, Tennessee
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✓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.

Abbreviations used in this paper:

EMA = epithelial membrane antigen; IPN = intraneural perineurioma; MR = magnetic resonance.

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