Intraneural perineurioma affecting multiple nerves: a case report and literature review

Int J Clin Exp Pathol. 2014 May 15;7(6):3347-54. eCollection 2014.


Intraneural perineurioma is a neoplasm of perineurial cells, corresponding to WHO grade I. We present a case of intraneural perineurioma affecting multiple nerves, which usually involved one or two of major nerve trunks in one patient. We describe the clinical presentation, magnetic resonance (MR) neurography characteristics, and pathological characteristics. The differential diagnosis with other diseases, such as neurofibroma, Schwannomatosis and HNPP, will also be discussed. We also review the literature in efforts to highlight recent studies on intraneural perineurioma and heighten and awareness for the possible presentations of this disorder.

Keywords: Intraneural perineurioma; MR; neuropathology; pseudo-onion bulbs.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Arthrogryposis / diagnosis
  • Diagnosis, Differential
  • Female
  • Hereditary Sensory and Motor Neuropathy / diagnosis
  • Humans
  • Nerve Sheath Neoplasms / pathology*
  • Neurilemmoma / diagnosis
  • Neurofibroma / diagnosis
  • Neurofibromatoses / diagnosis
  • Peripheral Nervous System Neoplasms / pathology*
  • Skin Neoplasms / diagnosis
  • Young Adult

Supplementary concepts

  • Schwannomatosis
  • Tomaculous neuropathy