Very long-term prognosis of patients with malignant uveal melanoma

PURPOSE. To investigate the very long-term prognosis of patients with uveal melanoma and the clinical characteristics influencing it. METHODS. Charts, registry data, and histopathologic specimens of 289 consecutive patients with choroidal And ciliary body melanoma treated in the district of the Helsinki University Central Hospital, Finland, between 1962 and 1981 were audited. Definitions for coding the cause of death were adapted from the Collaborative Ocular Melanoma Study (COMS). Competing risks were taken into account by using cumulative incidence analysis and competing risks regression. RESULTS. Of the 289 patients treated, 239 were deceased at the end of follow-up. The audited cause of death was uveal melanoma in 145 (61%) of them. The median follow-up of the 50 survivors was 28 years. The original histopathologic diagnosis of metastasis and second cancer was correct in 91% of all specimens, but immunohistochemical reassessment changed 10% of biopsy and 7% of autopsy diagnoses. Of 45 positive autopsies, 18% were performed without suspicion of melanoma. Uveal melanoma-related mortality was 31% (95% confidence interval [CI], 26-37) by 5 years, 45% (95% CI, 40-51) by 15 years, 49% (95% CI, 43-55) by 25 years, and 52% (95% CI, 45-58) by 35 years, according to cumulative incidence analysis. Of patients who died of uveal melanoma, 62%, 90%, 98%, and 100% did so within 5, 15, 25, and 35 years, respectively. Between 15 and 35 years, 20% to 33% of deaths were still due to uveal melanoma. By competing risks regression analysis, the hazard ratio was 1.08 (P = 0.0012) for each millimeter increase in tumor diameter, 2.27 (P = 0.0076) for extraocular growth, and 1.89 (P = 0.0011) for ciliary body involvement. CONCLUSIONS. Metastatic uveal melanoma was the leading single cause of death throughout the study. Cumulative incidences provide a sound basis for patient counseling and design of trials.