Ocular (Eye) Melanoma

by Dr. C.H. Weaver M.D. updated 1/2020

Ocular melanoma develops in the cells that produce eye color and occurs in or around the eye. Ocular melanoma is the most common cancer of the eye but only occurs in approximately 2500 individuals each year in the U.S. Ocular melanoma originates from mutated pigment cells but unlike skin melanoma, sun exposure is not the cause and ocular melanoma can occur in all races. Ocular melanoma is often diagnosed on a routine eye exam.

Signs & Symptoms of Ocular Melanoma can include:

  • Blurry vision or sudden loss of vision
  • Floaters (spots or squiggles drifting in the field of vision) or flashes of light.
  • Visual field loss (losing part of your field of sight)
  • A growing dark spot on the colored part of the eye (iris)

What causes ocular melanoma?

Individuals with light colored eyes are at higher risk of getting ocular melanoma and the majority of cases appear to develop from random mutations. Individuals who have had a melanoma skin cancer aren’t at a higher risk of developing an ocular melanoma.

Treatment of Ocular Melanoma

Treatment of the cancer can be accomplished with surgical removal of the eye or the use of radiation therapy. Both surgery and radiation appear equally effective but result in loss of vision. When the cancer is confined to the eye, the 5-year survival rate is 80 percent.

Overall 5-year survival for ocular melanoma once it has spread away from the eye is less than 10 percent. The most common location for the cancer cells to metastasize is directly into the bloodstream and typically to the liver.

Systemic therapy for metastatic ocular melanoma is relatively ineffective and newer checkpoint inhibitor medications that work well for the treatment of skin melanoma like Keytruda (pembrolizumab) and Opdivo (nivolumab) are not very effective. Patients with metastatic melanoma originating in the eye are candidates for clinical trials with newer agents that may show more promise than the currently available drugs.

Partial Chromosome 3 Deletion Tied to Poorer Survival in Uveal Melanomas

Patients with uveal melanomas carrying a partial deletion of chromosome 3 encompassing the BAP1 locus have a poor prognosis compared with patients whose uveal melanomas lack the deletion.

Manuel Rodrigues, MD, PhD, Institut Curie, PSL Research University, Paris, France, and colleagues conducted a retrospective genomic evaluation in individuals with uveal melanoma to evaluate the link between partial chromosome 3 deletion and metastasis-free survival (MFS).

Overall 4.0% of uveal melanomas were found to carry partial deletions of chromosome 3.

The 60-month MFS rate was were 33% for uveal melanomas that carried a deletion of the BAP1 locus compared to 81% for uveal melanomas lacking BAP1 locus deletion. The 60-month OS rates were 65% compared to 84%.

Deletion of chromosome 3 encompassing the BAP1 locus is associated with a poor prognosis and individuals with these deletions should explore more aggressive treatment options than those without. (1)

Treatment of intraocular or uveal melanoma is complex and well reviewed here: https://www.cancer.gov/types/eye/patient/intraocular-melanoma-treatment-pdq

Reference:

  1. JAMA Ophthalmol. 2020 Jan 2. Epub ahead of print.
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