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Melanoma pp 453-468 | Cite as

Ocular Melanoma

  • Armin R. Afshar
  • Bertil E. DamatoEmail author
  • Boris C. Bastian
Reference work entry

Abstract

Approximately 5% of all melanomas arise in the eye. Of these ocular melanomas, about 97% are of uveal origin and 3% are conjunctival. Uveal and conjunctival melanomas are biologically and clinically distinct diseases. Uveal melanomas are mostly initiated by GNAQ and GNA11 mutations and progress via SF3B1, EIF1AX, and BAP1 mutations, the latter greatly enhancing metastatic potential. Conjunctival melanomas are biologically similar to cutaneous melanomas, with common mutations of BRAF or NRAS as initiating mutations. The first choice of treatment for primary uveal melanomas is radiotherapy in most centers, whereas conjunctival melanomas are treated by excision with adjunctive radiotherapy and/or topical chemotherapy. With uveal melanomas, the prognosis for survival is mostly based on genetic studies, whereas with conjunctival melanomas tumor size and location are more predictive. There currently is no effective treatment for metastatic uveal melanoma, whereas conjunctival melanomas, like cutaneous melanomas, can be treated with targeted therapy and/or immunotherapy.

Keywords

Ocular melanoma Uveal melanoma Conjunctival melanoma Targeted therapy Immunotherapy Genetics Histology Diagnosis Treatment 

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Armin R. Afshar
    • 1
  • Bertil E. Damato
    • 2
    Email author
  • Boris C. Bastian
    • 3
  1. 1.Vitreoretinal Surgery and Ocular Oncology, Department of OphthalmologyUniversity of California, San FranciscoSan FranciscoUSA
  2. 2.Ocular Oncology Service, Departments of Ophthalmology and Radiation OncologyUniversity of California, San FranciscoSan FranciscoUSA
  3. 3.Departments of Dermatology and Pathology, Helen Diller Family Comprehensive Cancer CenterUniversity of California, San FranciscoSan FranciscoUSA

Section editors and affiliations

  • Boris Bastian
    • 1
  • Hensin Tsao
    • 2
    • 3
  1. 1.Departments of Dermatology and PathologyUniversity of California, San FranciscoSan FranciscoUSA
  2. 2.AuburndaleUSA
  3. 3.Harvard-MIT Health Sciences and TechnologyCambridgeUSA

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