Abstract

AbstractThe deadly natural history of uveal melanoma was fully described, unknowingly, in a well known English artist in 1792 and soon thereafter in an unknown Scottish woman around 1808. It became a well recognised entity much later, by 1868. Today, with the exception of being able to save the eye of their patient, ocular oncologists managing patients with uveal melanoma find themselves in essentially the same situation than their forebears: mortality rates have not noticeably decreased and metastatic melanoma continues to be the “hideous picture of disease” that it was 150 years ago. Metastatic melanoma is the single overwhelming cause of death in patients with uveal melanoma, and no consistently effective treatment is known for disseminated disease. One reason for this unhappy state of affairs is that patients formerly were dismissed after enucleation until they presented with advanced metastasis to an oncologist who did not recognise uveal melanoma as a disease very different from cutaneous melanoma. The advent of ocular oncology has led to rational early detection programs for subclinical metastasis, validated staging of metastatic disease, and first controlled clinical trials of managing metastases with therapies specifically aimed against this cancer. Basic research highlights uveal melanoma as a typically slowly growing, early metastasising cancer, and staging, grading and typing of primary tumours is leading to rational assignment of patients to follow‐up and adjuvant treatment trials, which hopefully will improve their survival rate. The current understanding is that, by the time the eye becomes symptomatic, uveal melanomas prone to metastasis already have seeded micrometastases, which need to be kept under control if we are to eventually conquer this disease.

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