Uveal Neural Tumors

  • Victoria M. L. Cohen
  • Bertil E. Damato
  • Arun D. SinghEmail author


Peripheral nerve sheath tumors of the uveal tract arise mainly in the sheath of peripheral nerves that are found in the uvea. The ciliary nerve is most often affected, and hence these tumors tend to be located anteriorly, in the ciliary body or peripheral choroid in the anatomic location of the long ciliary nerve. There are several systemic predisposing risk factors, which include schwannomatosis, neurofibromatosis 1 (NF1) and 2, and other rare genetic syndromes. The benign tumors can be subclassified as neurofibroma and schwannoma. The malignant nerve sheath tumors are more difficult to subclassify if they lose the histological characteristics of their benign counterparts. In this chapter, we discuss uveal neurofibroma and schwannoma.


Uveal neurofibroma Ganglioneurofibroma Uveal schwannoma 


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Victoria M. L. Cohen
    • 1
  • Bertil E. Damato
    • 2
  • Arun D. Singh
    • 3
    Email author
  1. 1.Department of Ocular OncologyMoorfields Eye HospitalLondonUK
  2. 2.Nuffield Department of Clinical Neurosciences, University of OxfordJohn Radcliffe HospitalOxfordUK
  3. 3.Department of Ophthalmic OncologyCole Eye Institute, Cleveland ClinicClevelandUSA

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