Abstract

Uveal melanoma is the most common intraocular tumor in adults. Metastatic disease occurs in about 30% of patients, for which there is currently no effective treatment. More than half of patients are long-term survivors, and it is well established that cancer survivors are prone to developing second primary cancers. In this study, we analyzed 10 years’ worth of data from the nationwide database to determine the rates of coexisting malignancies and second primary cancers associated with uveal melanoma. The mean annual incidence of uveal melanoma was 1.1 per million. Approximately 43% of patients had coexisting cancers. The most common coexisting cancer was lung cancer (10%) followed by liver cancer (6%) and non-Hodgkin lymphoma (6%). In patients whose first cancer in their lifetime was uveal melanoma, the 10-year cumulative incidence of second primary cancers was 22% (95% confidence interval, 9–31%). The age- and sex-adjusted standard incidence rates was 3.61 (95% confidence interval, 2.61–4.86). The most common second primary cancers were lung cancer and hepatocellular carcinoma, followed by prostate, thyroid, pancreatic, and ovarian cancers. Age was the only factor associated with second primary cancer development. Our findings will be helpful in providing counseling for cancer screening in uveal melanoma patients.

Highlights

  • Uveal melanoma is the most common intraocular tumor in adults [1,2]

  • Population-based study, we showed that >40% patients with uveal melanoma had one or more coexisting cancers

  • This is a higher rate than those in previous studies reporting that ~20% patients with uveal melanoma had coexisting cancers [20,21]

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Summary

Introduction

Uveal melanoma is the most common intraocular tumor in adults [1,2]. several of the signaling pathways involved in uveal melanoma have been discovered, this has not translated into an increase in survival rate [3,4,5,6,7]. Many cancer survivors have coexisting cancers and second primary cancers (SPCs), a phenomenon ascribed to both environmental factors, such as smoking and occupation, and genetic factors [10,11,12,13,14]. Patients with familial adenomatous polyposis frequently develop colon cancer, osteoma, adrenal carcinoma, and thyroid cancer due to the increased β-catenin activity caused by the APC gene mutation [15,16,17]. Women positive for the BRCA1/2 gene mutation frequently develop hereditary breast and ovarian cancers [18,19]. In this context, identifying cancers that accompany uveal melanoma could help to elucidate the underlying mechanism of uveal melanoma and enable counseling provisions for cancer screening

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