Optic nerve sheath meningioma in a patient with choroidal malignant melanoma: a case report of a complex neurocristopathy

Orbit. 2009;28(6):436-8. doi: 10.3109/01676830903180314.


Purpose: To describe the clinical findings in a patient who had developed choroidal melanoma and was incidentally found to have meningioma of the optic nerve in the same side.

Methods: Clinical and histopathological findings of the case are reviewed and presented.

Results: The patient had a choroidal melanoma of the left eye for which she had initially declined surgery and 2 years later when she underwent an examination, an optic nerve sheath meningioma was incidentally found histologic examination. Both tumours are derived from neural crest cells.

Conclusion: Complex neurocristopathy, a disorder resulting from aberrations in the growth and development of neural crest derived structures has been very rarely described in the eye and orbit. To the best of our knowledge there has been only one previous case report of a combination of neurofibroma in the right upper lid, meningioma in the right middle cranial fossa and uveal malignant melanoma in the left eye. (However in this patient the tumours were at 3 different sites.).

Publication types

  • Case Reports

MeSH terms

  • Aged, 80 and over
  • Choroid Neoplasms / pathology*
  • Fatal Outcome
  • Female
  • Gonioscopy
  • Humans
  • Intraocular Pressure
  • Melanoma / pathology*
  • Meningeal Neoplasms / pathology*
  • Meningioma / pathology*
  • Neoplasms, Second Primary*
  • Optic Nerve Neoplasms / pathology*
  • Tomography, X-Ray Computed