Abstract
Survival in uveal melanoma has remained unchanged since the early 1970s. Because outcomes are highly related to the size of the tumour, timely and accurate diagnosis can increase the chance for cure. A consensus-based guideline was developed to inform practitioners. PubMed was searched for publications related to this topic. Reference lists of key publications were hand-searched. The National Guidelines Clearinghouse and individual guideline organizations were searched for relevant guidelines. Consensus discussions by a group of content experts from medical, radiation, and surgical oncology were used to formulate the recommendations. Eighty-four publications, including five existing guidelines, formed the evidence base. Key recommendations highlight that, for uveal melanoma and its indeterminate melanocytic lesions in the uveal tract, management is complex and requires experienced specialists with training in ophthalmologic oncology. Staging examinations include serum and radiologic investigations. Large lesions are still most often treated with enucleation, and yet radiotherapy is the most common treatment for tumours that qualify. Adjuvant therapy has yet to demonstrate efficacy in reducing the risk of metastasis, and no systemic therapy clearly improves outcomes in metastatic disease. Where available, enrolment in clinical trials is encouraged for patients with metastatic disease. Highly selected patients might benefit from surgical resection of liver metastases.
Highlights
Survival in uveal melanoma has remained unchanged since the early 1970s
Monosomy 3, with a gain in chromosome 8q, and gep class 2 are associated with 3-year metastasis-free survival rates of 53% and 50% respectively[8,13,14]; these genetic variations occur in about 50% of patients[13,15,16,17,18]
Literature was identified for diagnosis and staging, observation, surgery, brachytherapy, transpupillary thermotherapy, management of metastatic disease, and follow-up
Summary
Survival in uveal melanoma has remained unchanged since the early 1970s. Because outcomes are highly related to the size of the tumour, timely and accurate diagnosis can increase the chance for cure. Factors associated with poor prognosis include large tumour size, tumour location in the ciliary body, intermediate or epithelioid cell type, proximity to the location of the tumour anterior margin, presence of extraocular extension, high mitotic rate, and lymphocytic infiltration[10,11,12]. Monosomy 3, with a gain in chromosome 8q, and gep class 2 are associated with 3-year metastasis-free survival rates of 53% and 50% respectively[8,13,14]; these genetic variations occur in about 50% of patients[13,15,16,17,18]. Subsequent work has demonstrated that tumour size (basal dimension) is e57 an independent predictor of survival for gep class 1 and 2 patients alike[19]
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