Hybrid neurofibroma/schwannoma of the orbit

Rev Esp Patol. 2023 Apr-Jun;56(2):140-143. doi: 10.1016/j.patol.2022.03.002. Epub 2022 Jun 15.

Abstract

Hybrid neurofibroma/schwannoma is a rare variant of hybrid peripheral nerve sheath tumours (HPNST). A Medline search up to December 2021 identified only six cases of this tumour in the orbit. We report the case of a 78-year-old man who presented with left exophthalmos. Computed tomography showed a left intraconal orbital mass. The clinico-radiological diagnosis was consistent with an intraconal cavernous angioma. Orbitotomy was performed, obtaining an 18×16×11mm mass. Two different morphologies were seen microscopically, diagnostic of hybrid neurofibroma/schwannoma. HPNSTs of the orbit are uncommon and most reported cases showed a hybrid neurofibroma/schwannoma morphology. Hybrid neurofibroma/schwannomas have been associated with neurofibromatosis and schwannomatosis. Local recurrences have been reported. The correct identification of these tumours is important due to their potential use as a syndromic marker.

Keywords: Neurofibroma; Peripheral nerve sheath tumour (PNST); Schwannoma; Tumores de la vaina del nervio periférico (PNST).

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Humans
  • Male
  • Nerve Sheath Neoplasms* / diagnosis
  • Nerve Sheath Neoplasms* / pathology
  • Neurilemmoma* / diagnostic imaging
  • Neurilemmoma* / pathology
  • Neurofibroma* / pathology
  • Neurofibromatoses*
  • Orbit / pathology