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      Additional primary malignancies in a Polish cohort of uveal melanoma patients: a review of 644 patients with long-term follow-up

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          Abstract

          Aim

          To investigate the frequency and location of additional primary malignancies in a Polish cohort of uveal melanoma (UM) patients registered in a single centre database.

          Material and method

          Retrospective data analysis of patients treated for uveal melanoma at the Department of Ophthalmology, Poznań University of Medical Sciences, Poland between 1991 and 2017. Data on the diagnosis of the additional malignancies were obtained during the follow-up visits in our Department and/or from the Greater Poland Cancer Registry. The exclusion criteria comprised no confirmed follow-up or incomplete clinical entry data.

          Results

          Among 644 UM patients registered in the database up to 2017, the additional malignancy was diagnosed in 126 (20%) patients: 71 men, 55 women at the median age of 67 years (range: 34–94). In 48 patients (38%), the additional malignancy occurred prior to the diagnosis of UM, in 73 (58%) patients - after it. The most common locations of second cancer were skin (20 cases / 15%), breast (17 cases / 13%) and lungs (15 cases / 12%). The median follow-up was 36 months (range: 3–242). 87 patients (69%) died by the study close, 32 (37%) of them due to metastatic disease from uveal melanoma, 41 (47%) due to another cancer.

          Conclusions

          The frequency of additional primary malignancies was higher in our cohort than reported by most of other groups. If there is a certain predisposition to a specific type of additional primary carcinoma in UM patients, the analysis of larger database is required.

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          Most cited references32

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          Uveal melanoma: trends in incidence, treatment, and survival.

          To determine trends in incidence, treatment, and survival with primary uveal melanoma in the United States over a 36-year period from 1973 to 2008. Systematic review of existing databases. A total of 4070 patients with primary uveal melanoma (International Classification of Disease for Oncology [ICD-O-2] codes C69.3 [choroid], C69.4 [ciliary body and iris], and C69.2 [retina]) derived from the Surveillance, Epidemiology, and End Results (SEER) program database in the United States from 1973 to 2008. The significance of trends in age-adjusted incidence, treatment, and 5-year relative survival rates were determined using chi-square testing and 95% confidence intervals (CIs). Age-adjusted incidence, form of treatment (surgery, radiation, or both), and 5-year relative survival rates. There were 4070 cases of uveal melanoma representing 3.1% of all recorded cases of melanoma. The majority of cases (98.3%) were reported by hospital inpatient/outpatient clinics. Histopathologic confirmation was available in 2804 cases (72.1% for all years). The mean age-adjusted incidence of uveal melanoma in the United States was 5.1 per million (95% CI, 4.8-5.3). The majority of cases (97.8%) occurred in the white population. There was a statistically significant variation of age-adjusted incidence between sexes (male = 5.8, 95% CI, 5.5-6.2; and female = 4.4, 95% CI, 4.2-4.7). A decreasing trend was observed in patients treated with surgery alone (93.8% for 1973-1975 vs. 28.3% for 2006-2008), whereas a corresponding increase was seen in those treated with radiation (1.8% for 1973-1975 vs. 62.5% for 2006-2008). No change in the 5-year relative survival rate (81.6%) was observed from 1973 to 2008. The age-adjusted incidence of uveal melanoma (5.1 per million) has remained unchanged from 1973 to 2008. Despite a shift toward more conservative treatments, survival has not improved during this time period. Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.
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            Uveal melanoma

            Uveal melanoma (UM) is the most common primary intraocular malignancy in adults. UMs are usually initiated by a mutation in GNAQ or GNA11, unlike cutaneous melanomas, which usually harbour a BRAF or NRAS mutation. The annual incidence in Europe and the USA is ~6 per million population per year. Risk factors include fair skin, light-coloured eyes, congenital ocular melanocytosis, ocular melanocytoma and the BAP1-tumour predisposition syndrome. Ocular treatment aims at preserving the eye and useful vision and, if possible, preventing metastases. Enucleation has largely been superseded by various forms of radiotherapy, phototherapy and local tumour resection, often administered in combination. Ocular outcomes are best with small tumours not extending close to the optic disc and/or fovea. Almost 50% of patients develop metastatic disease, which usually involves the liver, and is usually fatal within 1 year. Although UM metastases are less responsive than cutaneous melanoma to chemotherapy or immune checkpoint inhibitors, encouraging results have been reported with partial hepatectomy for solitary metastases, with percutaneous hepatic perfusion with melphalan or with tebentafusp. Better insight into tumour immunology and metabolism may lead to new treatments.
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              Incidence of uveal melanoma in Europe.

              To estimate incidence rates of uveal melanoma in Europe from 1983 to 1994. Incidence analysis of data from cancer registries adhering to the European Cancer Registry-based study on survival and care of cancer patients (EUROCARE) (cases diagnosed from 1983 to 1994). Data of 6673 patients with ocular melanoma (as defined by International Classification of Diseases for Oncology morphology codes 8720 to 8780 [melanoma] and International Classification of Diseases 9 (ICD9) codes 190.0 [iris and ciliary body], 190.5 [retina], 190.6 [choroid], and 190.9 [unspecified ocular location]) from 33 cancer registries of 16 European countries. Incidence rate ratios (IRRs) were obtained from a multilevel Poisson regression model. Incidence rates and IRRs associated with demographic and geographic variables. Standardized incidence rates increased from south to north across registries, from a minimum of 8 per million in Norway and Denmark. The inclusion of tumors with unspecified ocular location (code 190.9) increased incidence rates in most United Kingdom registries, but not in the other geographic areas, where this code was seldom used for uveal melanomas. Incidence increased noticeably up to age 55 (IRR, 1.46 per 5 years; 95% confidence interval [CI], 1.36-1.57) but leveled off after age 75 (IRR, 0.99 per 5 years; 95% CI, 0.93-1.05), with intermediate levels midway (IRR, 1.18 per 5 years; 95% CI, 1.12-1.23). It was also higher in males (IRR, 1.22; 95% CI, 1.16-1.28). Rates were stable during the study period, but a cohort effect was evidenced, accounting for higher incidence rates in people born during the period 1910 to 1935 (P = 0.005). Incidence increased with latitude (P = 0.008), which explained most differences in rates among areas. In this large series of uveal melanomas, we found stable incidence during the years 1983 to 1994. The north-to-south decreasing gradient supports the protective role of ocular pigmentation. European ophthalmologists should develop guidelines to standardize the coding of tumors treated conservatively using the ICD classification to improve the registration and surveillance of uveal melanoma by cancer registries.
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                Author and article information

                Contributors
                irospondkubiak@ump.edu.pl
                Journal
                BMC Ophthalmol
                BMC Ophthalmol
                BMC Ophthalmology
                BioMed Central (London )
                1471-2415
                12 December 2023
                12 December 2023
                2023
                : 23
                : 506
                Affiliations
                [1 ]Department of Ophthalmology, Poznań University of Medical Sciences, ( https://ror.org/02zbb2597) Poznań, Poland
                [2 ]Polyclinic of Plastic and Ophthalmic Surgery, Kobylniki, Poland
                [3 ]Department of Ophthalmology, Poznań District Hospital, Poznań, Poland
                [4 ]Greater Poland Cancer Registry, Greater Poland Cancer Centre, ( https://ror.org/0243nmr44) Poznań, Poland
                [5 ]Department of Food Safety and Quality Management, Poznań University of Life Sciences, ( https://ror.org/03tth1e03) Poznań, Poland
                Article
                3246
                10.1186/s12886-023-03246-z
                10717148
                38087265
                def767a2-b0ca-421a-af39-33fd96ae74e5
                © The Author(s) 2023

                Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                : 25 June 2023
                : 26 November 2023
                Categories
                Research
                Custom metadata
                © BioMed Central Ltd., part of Springer Nature 2023

                Ophthalmology & Optometry
                eye,malignant neoplasm,uveal melanoma,second primary carcinoma
                Ophthalmology & Optometry
                eye, malignant neoplasm, uveal melanoma, second primary carcinoma

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