Melanoma pp 273-312 | Cite as

Uveal Melanoma

  • Ronald W. MilamJr.
  • Anthony B. DanielsEmail author


Uveal melanoma, the most common primary intraocular malignancy, includes melanomas of the iris, ciliary body, and choroid. Uveal melanoma is distinctly different from cutaneous melanoma in its clinical and molecular features as well as its risk factors, genetics, pathogenesis, metastatic behavior, and treatment. The rate of distant metastasis approaches 50%, with the most common site of initial metastasis being the liver. Once metastatic disease develops, it is universally fatal, as there are currently no truly effective treatment options for metastatic disease. There are a number of host susceptibility factors and a limited number of well-conserved genetic derangements associated with uveal melanoma, including derangements of chromosomes 1, 3, 6, and 8 and mutations in the GNAQ, GNA11, SF3B1, EIF1AX, and BAP1 genes. Advanced genetic techniques, such as gene expression profiling (GEP) and multiplex ligand-dependent probe amplification (MLPA), have enhanced our ability to stratify patients into low and high risk for the development of metastatic disease. The diagnosis of uveal melanoma is clinical, with numerous adjuvant ophthalmic imaging techniques available to assist in distinguishing borderline benign lesions from melanoma. Currently, the vast majority of uveal melanomas are treated with globe-conserving radiotherapy (predominantly brachytherapy), with local control rates >95%. Enucleation is reserved for larger melanomas, although even these can be treated with various advanced forms of external radiotherapy. Despite successful and durable treatment of the primary tumor, long-term survival rates remain poor. This is likely due to micrometastatic disease that is not clinically or radiographically identified, and indeed present, at the time of initial diagnosis of the primary tumor. These micrometastases have the ability to remain dormant for many years, as patients may develop overt metastatic disease even decades after their initial treatment. Future studies that focus upon the earlier detection, prevention, and treatment of metastatic disease are greatly needed.


Uveal melanoma Ocular melanoma Choroidal melanoma Iris melanoma Ciliary body melanoma Risk factors Prognosis Genetics GEP MLPA Radiotherapy Brachytherapy Radiation retinopathy 


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Ophthalmology and Visual SciencesVanderbilt University Medical Center, Vanderbilt Eye InstituteNashvilleUSA
  2. 2.Department of Ophthalmology and Visual Sciences, Department of Radiation OncologyVanderbilt-Ingram Cancer Center, Vanderbilt University Medical CenterNashvilleUSA
  3. 3.Program in Cancer BiologyVanderbilt UniversityNashvilleUSA

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