Uveal melanoma in Finnish patients with congenital ocular melanocytosis

PurposeTo described the characteristics of uveal melanoma in congenital ocular melanocytosis in Finland.MethodsFor this retrospective observational case series, we identified all 15 of 867 (1.7%; 95% CI 1.0–2.8) uveal melanoma patients (5 male, 10 females) with congenital ocular melanocytosis during January 2000‐May 2015 at the Ocular Oncology Service, Helsinki University Hospital. The patient was followed up at 3 and 6 months, every 6 months for 3 years, and thereafter annually. The diagnosis of uveal melanoma was based on clinical and ultrasonographic features, and metastases were screened with annual abdominal ultrasonography and liver function tests.ResultsThe mean age at diagnosis was 55 years as compared with 64 years for all 867 patients. The mean thickness and basal diameter of the tumours were 6.5 (SD 3.6) and 13.1 (SD 4.4) mm, respectively, as compared to 5.1 (SD 3.9) and 12.5 (SD 4.5) among all patients, respectively. The melanoma involved the ciliary body in 6 patients (40%). The tumours represented TNM stages I in 4 (27%) patients, II in 5 (33%), III in 5 (33%) and IV (metastases at the time of diagnosis) in 1 patient. Enucleation was done in 2 cases, I‐125‐brachytherapy in 7 cases, and 5 patients were treated with Ru‐106. Recurrent tumour growth was observed in 2 patients. Overall 5‐ and 10‐year survival was 68% (95% CI 35–87) and 13% (95% CI 1–43). Survival was associated with TNM stage: 100%, 80%, 33% and 0% at 5 eyars for stages I‐IV, respectively. One patient survived for 10 years without metastasis.ConclusionsIn our data set, uveal melanoma patients with congenital ocular melanocytosis are on average 9 years younger than uveal melanoma patients in general, and have a tendency to die of metastases.