Abstract

BackgroundUveal melanoma (UM) is a rare but aggressive cancer, which is the most common primary intraocular malignancy in adults. We aimed to develop and validate a competing risk nomogram to predict cancer-specific survival (CSS) of patients with UM, as well as compare its prognostic value with that of the American Joint Committee on Cancer (AJCC) staging system.MethodsData of patients diagnosed with UM from 2010 to 2015 were identified from the Surveillance, Epidemiology, and End Results (SEER) database. We extracted and integrated significant prognostic factors based on competing risk regression to build a nomogram. The nomogram with an online prediction version was also created. The performance of the nomogram was evaluated using Harrell’s concordance index (C-index) and calibration plots. Receiver operating characteristic (ROC) curve was carried out to estimate clinical applicability of the model. Improvements in the predictive accuracy of our new model compared with AJCC staging system were estimated by calculating the relative integrated discrimination improvement (IDI) and the net reclassification improvement (NRI).ResultsA total of 839 eligible patients with primary UM were randomly assigned to a training cohort (588, 70%) and a validation cohort (251, 30%). Age, histological type, T stage and M stage were independent prognostic factors to predict CSS of UM and were incorporated in the nomogram. The calibration plots indicated that the 3- and 5-year CSS probabilities were consistent between the nomogram prediction and the actual observation. The C-index for this model was 0.778 (95% CI:0.756–0.800) and 0.786 (95% CI: 0.749–0.816) in the training cohort and validation cohort. Areas under the curve (AUCs) were 0.814, 0.771, and 0.792 in the training cohort, 0.788, 0.781 and 0.804 in the validation cohort, respectively. The NRI value in AJCC staging system was − 0.153 (95% CI -0.29 – − 0.041) for 3 years of follow-up and − 0.276 (95% CI -0.415 – − 0.132) for 5 years of follow-up. The IDI values for 3 and 5 years of follow-up in the AJCC staging system were − 0.021 (P = 0.076) and − 0.045 (P = 0.004), respectively.ConclusionsWe have developed and validated a competing risk nomogram to reliably predict cancer-specific survival of patients with UM. This convenient tool may be useful for evaluating cancer-specific prognosis.

Highlights

  • Uveal melanoma (UM) is a rare but aggressive cancer, which is the most common primary intraocular malignancy in adults

  • Five-year survival rates of UM were reported to be stable as approximately 80% in the past three decades, up to 50% of patients may develop metastases [3, 4], about 50% of patients with uveal melanoma might succumb to metastasis within 10 years of diagnosis

  • Patient characteristics A total of 839 eligible patients with primary UM were identified from the SEER database and included in the analysis

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Summary

Introduction

Uveal melanoma (UM) is a rare but aggressive cancer, which is the most common primary intraocular malignancy in adults. We aimed to develop and validate a competing risk nomogram to predict cancer-specific survival (CSS) of patients with UM, as well as compare its prognostic value with that of the American Joint Committee on Cancer (AJCC) staging system. Uveal melanoma (UM) is the most common primary intraocular malignancy in adults, with the mean ageadjusted incidence of 5.1 per million in the USA [1], representing up to 85% of ocular melanomas [2]. There have been a few articles reporting the incidence, treatment and survival of UM, the clinicopathological characteristics and prognosis of UM still need further study because of its low prevalence rate and limited cases in previous studies. The staging system for UM was changed to American Joint Committee on Cancer (AJCC) seventh edition since

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