Uveal melanoma natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Complications

Complications of eye melanoma may include:

Glaucoma

Eye melanoma that is growing may cause glaucoma. Signs and symptoms of glaucoma may include the following:

• Eye pain
• Redness
• Blurry vision

Vision loss

Eye melanomas that are large often cause vision loss in the affected eye and may cause complications, such as retinal detachment, that also cause vision loss. If small eye melanomas occur in critical parts of the eye they may cause some vision loss. There may be difficulty seeing in the center of the vision or on the side. Advanced eye melanomas can cause complete vision loss.

Metastasis

Eye melanoma can spread outside of the eye and to distant areas of the body, including the liver, lungs and bones.

Prognosis

Poor prognostic factors for systemic disease include:

• Older age: >60 years of age
• Larger tumors
• Anterior location within the globe
• Epithelioid cells (i.e. mixed or epithelioid histology)
• Extraocular extension
• Systemic metastases may be wide spread, with the liver > lung > bone > kidney > brain being a common sites for tumor deposits.
• Cell type is the most commonly used predictor of outcome following enucleation, with spindle-A cell melanomas carrying the best prognosis and epithelioid cell melanomas carrying the least favorable prognosis. Nevertheless, most tumors have an admixture of cell types, and there is no clear consensus regarding the proportion of epithelioid cells that constitutes designation of a tumor as mixed or epithelioid.
• Overall survival depends on tumour size, extraocular spread, and metastases. Even small (<10 mm diameter, <3 mm thickness) tumors still carry a 10-15% 5-year mortality.
• Several additional microscopic features can affect the prognosis of intraocular melanoma, including:
  • Mitotic activity.
  • Lymphocytic infiltration.
  • Fibrovascular loops
  • Extraocular extension, recurrence, and metastasis are associated with an extremely poor prognosis, and long-term survival cannot be expected.
  • The 5-year mortality rate associated with metastasis from ciliary body or choroidal melanoma is approximately 30%, compared with a rate of 2% to 3% for iris melanomas.

Uveal melanomas arise from melanocytes in the uvea. These melanomas have significant differences from cutaneous melanomas that guide their management [1].

Uveal melanomas can develop in any part of the uveal tract, including the iris, ciliary body, and choroid. Iris melanomas are less common than choroidal melanomas (approximately 5 versus more than 80 percent of cases). Iris melanomas are also associated with a better prognosis than ciliochoroidal tumors (10 year-survival approximately 95 versus 77 percent). Because of the less aggressive nature of iris melanomas, these tumors are usually managed conservatively (ie, close monitoring of the tumor). Fast-growing tumors may be excised. This topic will emphasize the management of uveal melanoma as it pertains to choroidal and ciliary body melanoma, the more common and life-threatening forms of the disease.