Hybrid Neurofibroma/Schwannoma of the Oral Cavity: A Rare Case Report and Literature Review

Int J Surg Pathol. 2023 Aug;31(5):695-701. doi: 10.1177/10668969221117978. Epub 2022 Aug 15.


Hybrid peripheral nerve sheath tumors show combined features of more than one type of conventional benign peripheral nerve sheath tumors. There are few cases reported of hybrid peripheral nerve sheath tumors in the head and neck region. A 68-year-old female patient was referred for evaluation of an oral swelling lasting five years. Intraoral examination revealed a small mobile nodule located in the lower vestibule. The patient underwent excisional biopsy and microscopic evaluation showed typical features of neurofibroma enclosing areas with palisading nuclei compatible with Antoni A pattern, which are seen in schwannomas. These regions showed strong and diffuse immunoreactivity for S100 protein and moderate positivity in the neurofibroma area. CD34 was positive in the neurofibroma area and entrapped axons were positive for neurofilament. The final diagnosis was oral hybrid neurofibroma-schwannoma tumor. Hybrid peripheral nerve sheath tumors, although extremely rare, may arise within the oral cavity. To the best of our knowledge, this is the first neurofibroma-schwannoma tumor reported in the oral cavity. Recognizing hybrid peripheral nerve sheath tumors as a distinct clinicopathological entity is important because they may also be associated with syndromic disorders.

Keywords: hybrid neurofibroma-schwannoma tumor; hybrid peripheral nerve sheath tumors; neurofibromatosis; oral cavity; schwannomatosis; syndromic disorders.

Publication types

  • Review
  • Case Reports

MeSH terms

  • Aged
  • Brain Neoplasms*
  • Female
  • Humans
  • Mouth Neoplasms* / diagnosis
  • Mouth Neoplasms* / surgery
  • Nerve Sheath Neoplasms* / diagnosis
  • Nerve Sheath Neoplasms* / pathology
  • Nerve Sheath Neoplasms* / surgery
  • Neurilemmoma* / diagnosis
  • Neurilemmoma* / surgery
  • Neurofibroma* / diagnosis
  • Neurofibroma* / pathology
  • Neurofibroma* / surgery
  • S100 Proteins


  • S100 Proteins