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]
Overview
If left untreated, 50% of patients with uveal melanoma may progress to develop metastasis. Common complications of uveal melanoma include glaucoma, vision loss, and metastasis. Prognosis is generally good for patients with iris melanoma, and the 10 year-survival for iris melanoma is approximately 95 percent and for ciliochoroidal tumors is 77 percent respectively.
Natural History, Complications, and Prognosis
Natural history
- The clinical course is unpredictable and metastatic disease can develop very late after a long disease-free interval. [1][2]
- Uveal melanoma metastasizes haematogenously, predominantly to the liver.
- Less than 4% of patients with uveal melanoma have detectable metastatic disease, at the time of diagnosis.
- However in further course about half of patients will develop metastases.
- When metastatic disease appears it unavoidably leads to death because of lack of effective systemic treatment.
Complications
Complications of eye melanoma include the following:[3]
- Glaucoma
- Cataract
- Anterior chamber hemorrhage
- Corneal decompensation with edema and band keratopathy
- Metastasis
- 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.
Complications of therapy of uveal melanoma
- Complications of radiotherapy include the following:[4]
- Radiation retinopathy
- Radiation maculopathy
- Radiation opticopathy
- Maculopathy
- Cataract
- Neovascular glaucoma
- Vitreous haemorrhage
- Retinal detachment and dryness
- Radiation retinopathy, opticopathy and neovascular glaucoma are responsible for the majority of secondary visual loss and secondary enucleations after therapy.
Prognosis
- 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.[5][6]
- Overall survival depends on tumor size, extraocular spread, and metastases. Even small (<10 mm diameter, <3 mm thickness) tumors still carry a 10-15% 5-year mortality.
- The 10 year-survival for iris melanoma is approximately 95 percent and for ciliochoroidal tumors is 77 percent respectively.
- The tumor node metastasis (TNM) staging system of the American Joint Committee on Cancer takes into account the key factors involved which are known to be of prognostic significance.
- The prognosis is usually poor when local control is not achieved with the initial treatment.[7][8]
- Detection of circulating tumor cells at the time of diagnosis is an independent risk factor for relapse and shortened survival in patients at high-risk based upon clinical parameters.[9][10]
- The prognosis for any patient with recurring or relapsing disease is poor, regardless of cell type or stage.