Objective: To study the clinical profile of young patients with uveal melanoma.
Design: Retrospective case-control series.
Setting: Tertiary referral center.
Patients: Data on 63 patients aged 20 years or younger with uveal melanoma were reviewed for clinical profile and association with oculo(dermal) melanocytosis, familial uveal melanoma, dysplastic nevus syndrome, cutaneous melanoma, and other second malignant neoplasms.
Results: Of 8000 patients with uveal melanoma, 63 (0.8%) were found in patients who were 20 years of age or younger. The median age at diagnosis was 16 years, and the youngest patient was 3 years old. Sixty-two patients (98%) were white, and uveal melanoma was unilateral in all cases. Seven patients (11%) had oculo(dermal) melanocytosis. Two patients (3%) had dysplastic nevi syndrome, and personal history of cutaneous melanoma was observed in 1 patient (2%). No other second cancers were present in any patient. The 5- and 15-year posttreatment survival estimates were 0.95 (95% confidence interval, 0.87-1.00) and 0.77 (95% confidence interval, 0.52-1.00), respectively.
Conclusions: Uveal melanoma is rare in children or teenagers. It occurs in a heterogeneous group displaying various associations, especially with oculo(dermal) melanocytosis. Oculo(dermal) melanocytosis is 9 times (95% confidence interval, 3.6-22.8) more common in young patients with uveal melanoma than in the general population with uveal melanoma. Young patients with uveal melanoma have short-term (5-year) survival better than that of adults, but the long-term (15-year) survival is similar to that of adults. Arch Ophthalmol. 2000;118:918-923