http://pubs.jamanetwork.com/ en-us Thu, 16 May 2013 00:00:00 GMT Thu, 16 May 2013 15:45:15 GMT Silverchair editor@pubs.jamanetwork.com webmaster@pubs.jamanetwork.com http://pubs.jamanetwork.com/article.aspx?articleID=1687776 Thu, 16 May 2013 00:00:00 GMT Shields CL, Kaliki S, Livesey M, et al. <span class="paragraphSection"><div class="boxTitle">Importance</div>Ocular/oculodermal (oculo[dermal]) melanocytosis is a congenital periocular pigmentary condition that can lead to the development of uveal melanoma, estimated at 1 in 400 affected patients. In this study, patients with melanocytosis who developed uveal melanoma were found to have double the risk for metastasis compared with those without melanocytosis.<div class="boxTitle">Objective</div>To determine the relationship of oculo(dermal) melanocytosis to the prognosis of patients with uveal melanoma.<div class="boxTitle">Design, Setting, and Patients</div>Retrospective chart review of 7872 patients with uveal melanoma treated at the Ocular Oncology Service, Wills Eye Institute, from August 25, 1970, through August 27, 2008.<div class="boxTitle">Exposures</div>Enucleation, plaque radiotherapy, local resection, or thermotherapy.<div class="boxTitle">Main Outcomes and Measures</div>Metastasis and death.<div class="boxTitle">Results</div>Of 7872 patients with uveal melanoma, oculo(dermal) melanocytosis was present in 230 (3%). The melanocytosis involved the sclera (92%), iris (17%), choroid (12%), eyelid (8%), and temporal fossa (1%). Eyes with melanoma and oculo(dermal) melanocytosis had a relative risk for metastasis 1.6 times greater compared with those with no melanocytosis (P < .001). Metastasis of uveal melanoma was 2.8 times higher in patients with iris melanocytosis (P < .001), 2.6 times higher with choroidal melanocytosis (P = .02), and 1.9 times higher with scleral melanocytosis (P < .001). By Kaplan-Meier estimates, metastasis in patients with oculo(dermal) melanocytosis vs no melanocytosis was 2% vs 1.8% at 1 year, 27% vs 15% at 5 years, and 48% vs 24% at 10 years (P < .001). By multivariable analysis, the factors predictive of metastasis in patients harboring uveal melanoma associated with oculo(dermal) melanocytosis were increased tumor thickness (P = .001) and the presence of subretinal fluid (P = .05), and the only factor predictive of death was increased tumor thickness (P = .009).<div class="boxTitle">Conclusions and Relevance</div>Patients with uveal melanoma associated with oculo(dermal) melanocytosis have double the risk for metastasis compared with those with no melanocytosis. All patients with oculo(dermal) melanocytosis should undergo ophthalmic examination and imaging on a twice-yearly basis because this could help with the early detection of melanoma.</span> 1 9 10.1001/jamaophthalmol.2013.129 http://pubs.jamanetwork.com/article.aspx?articleID=1687776