Abstract
Uveal melanoma (UM) is the most prevalent primary intraocular malignancy in adults with a stable incidence rate between five and seven cases per million in Europe and the United States. Although UM and melanoma from other sites have the same origin, UM has different epidemiological, biological, pathological and clinical features including characteristic metastatic hepatotropism. Despite improvements in the treatment of primary tumours, approximately 50% of patients with UM will develop metastases. In 90% of cases the liver is the first site of metastasis, however the mechanisms underlying this hepatic tropism have not been elucidated. Metastatic disease is associated with a very poor prognosis with a median overall survival of 6 to 12 months. Currently, there is no standard systemic treatment available for metastatic UM and once liver metastases have developed, prognosis is relatively poor. In order to prolong survival, close follow-up in all patients with UM is recommended for early detection and treatment. The treatment of metastatic UM includes systemic chemotherapy, immunotherapy and molecular targeted therapy. Liver-directed therapies, such as resection, radioembolization, chemoembolization, immunoembolization, isolated and percutaneous liver perfusion as well as thermal ablation represent available treatment options. However, to date a consensus regarding the optimal method of treatment is still lacking and the importance of setting guidelines in the treatment and management of metastatic UM is becoming a priority. Improvement in knowledge and a better insight into tumour biology, immunology and metastatic mechanism may improve current treatment methods and lead to the development of new strategies paving the way for a personalized approach.
Highlights
Uveal melanoma (UM) relatively rare is the most common primary intraocular malignancy in adults representing approximately 3–5% of all melanoma and 80% of ocular melanoma cases [1,2,3]
It has been hypothesized that high incidence of UM hepatic metastases may be caused by increased expression of cMET, a tyrosine kinase inhibitor activated by binding to the hepatic growth factor (HGF) receptor produced in the liver which is elevated in primary UMs [21,77]
The attempt to treat with a combination of cytotoxic T-lymphocyte associated protein 4 (CTLA-4) and PD-1 blockade failed to show significant clinical benefit in UM; it was shown to be associated with the considerable side effects [130]
Summary
Uveal melanoma (UM) relatively rare is the most common primary intraocular malignancy in adults representing approximately 3–5% of all melanoma and 80% of ocular melanoma cases [1,2,3]. Despite significant improvement in local treatment of the primary tumour, the 5-year survival rate of patients with UM is 50–70% and remains unchanged over the past decades [5,8,18,19,20]. 50% of the patients develop metastases within five years from initial diagnosis with median survival between 6 and 12 months due to the lack of efficient treatment options [9,21,22,23,24,25]. Advancement in the understanding of UM biological behaviour, the molecular and immunobiological characteristics are central to the development of new therapeutic strategies which will enable new therapeutic goals and approaches to treatment bringing benefit to patients with metastatic UM
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