IMPORTANCE: 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.
OBJECTIVE: To determine the relationship of oculo(dermal) melanocytosis to the 
prognosis of patients with uveal melanoma.
DESIGN, SETTING, AND PATIENTS: 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.
EXPOSURES: Enucleation, plaque radiotherapy, local resection, or thermotherapy.
MAIN OUTCOMES AND MEASURES: Metastasis and death.
RESULTS: 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). CONCLUSIONS AND RELEVANCE 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.