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The cancer risk in Marfan syndrome is not fully understood, but recent reports have suggested an increased risk in adults. This study assessed cancer risk in Marfan syndrome using population\u2010based data from the Swedish National registers.<\/jats:p>\n                  <jats:p>We performed a register\u2010based nationwide matched cohort study of 1544 individuals with Marfan syndrome, born 1930\u20132017. Each individual was matched to 50 comparisons by birth year, sex and birth county. Cancer risk was estimated using Cox proportional hazard models, expressed as hazard ratios (HRs) with 95% confidence intervals (CIs). All cancer cases diagnosed before age 20\u2009years were classified as childhood cancer, and those diagnosed at age 20\u2009years or older were classified as adult cancer.<\/jats:p>\n                  <jats:p>The overall risk of adult cancer was not increased in individuals with Marfan syndrome (HR 1.00, 95% CI 0.78\u20131.27). However, there was an increased risk of endocrine tumours in adults with Marfan syndrome (HR 2.86, 95% CI 1.40\u20135.85). Furthermore, an over two\u2010fold increased risk of cancer was observed among children with Marfan syndrome (HR 2.44, 95% CI 1.25\u20134.80).<\/jats:p>\n                  <jats:p>In this study, we found an increased cancer risk in children with Marfan syndrome. In contrast to previous reports, we did not detect an increased overall cancer risk in adults with Marfan syndrome, but an elevated site\u2010specific risk of endocrine tumours. Further, larger studies are warranted to evaluate the lifetime cancer risk in Marfan syndrome at different ages.<\/jats:p>","DOI":"10.1002\/ijc.70541","type":"journal-article","created":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T04:44:12Z","timestamp":1778733852000},"page":"1707-1714","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Cancer Risk in Marfan Syndrome: A Swedish Population\u2010Based Cohort Study"],"prefix":"10.1002","volume":"159","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-3000-361X","authenticated-orcid":false,"given":"Ida","family":"Nordgren","sequence":"first","affiliation":[{"name":"Department of Molecular Medicine and Surgery Karolinska Institutet  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna Skarin","family":"Nordenvall","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Surgery Karolinska Institutet  Stockholm Sweden"},{"name":"Department of Radiology Karolinska University Hospital  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Wachtmeister","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Surgery Karolinska Institutet  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fulya","family":"Taylan","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Surgery Karolinska Institutet  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yunxia","family":"Lu","sequence":"additional","affiliation":[{"name":"Department of Population Health and Disease Prevention &amp; Department of Epidemiology and Biostatistics, Joe. 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