Multiple orbital neurofibromas unassociated with von Recklinghausen's disease

Arch Ophthalmol. 1990 Jan;108(1):80-3. doi: 10.1001/archopht.1990.01070030086034.


A 58-year-old man with a 10-year history of right periorbital pain treated with multiple analgesics presented with slowly progressive exophthalmos of the right eye. Orbital imaging studies disclosed three separate well-defined tumors, located in the temporal fossa, the intraconal space, and within the floor of the orbit. All three tumors were removed intact and proved on histopathologic evaluation to be localized neurofibromas. The patient had complete relief of the chronic pain following removal of the tumors. We emphasize that the unusual occurrence of multiple circumscribed orbital tumors should suggest the diagnosis of neurofibroma even in the absence of von Recklinghausen's neurofibromatosis.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Humans
  • Male
  • Middle Aged
  • Neurofibroma / diagnosis*
  • Neurofibroma / pathology
  • Neurofibroma / surgery
  • Neurofibromatosis 1 / diagnosis*
  • Neurofibromatosis 1 / pathology
  • Neurofibromatosis 1 / surgery
  • Orbital Neoplasms / diagnosis*
  • Orbital Neoplasms / pathology
  • Orbital Neoplasms / surgery
  • Tomography, X-Ray Computed