Developments in the management of uveal melanoma.

Uveal melanomas threaten visual loss, enucleation, and death from metastatic disease. Most patients present because of symptoms, but failures of detection and diagnosis still occur commonly. Treatments aimed at avoiding enucleation include: plaque, proton beam or stereotactic radiotherapy; trans-scleral or trans-retinal local resection; and transpupillary thermotherapy. Increasingly, different modalities are being used in combination. The ocular outcomes are related to tumour size, location, spread and cell type. Metastatic disease occurs in many patients and is related to factors such as tumour dimensions, ciliary body involvement, cell type, extravascular matrix patterns and cytogenetics. Abnormalities related to chromosomes 3, 6 and 8 are strongly related to tumour behaviour, for the first time enabling survival probability to be estimated with a high degree of reliability in an individual patient. This enables high-risk individuals to be targeted for screening while providing reassurance to those with a minimal chance of developing metastatic disease. Such targeting would allow selection of patients for adjuvant systemic therapy, should a suitable treatment become available, and would also facilitate the evaluation of such treatment by increasing the statistical power of any randomized prospective study. The high mortality in patients with monosomy 3 melanoma suggests that in these patients ocular treatment is only palliative. Cytogenetic studies suggest that some melanomas may never develop any metastatic potential and if these impressions are confirmed by further studies, then in these patients the main priority of treatment would be to conserve vision.