{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T17:35:27Z","timestamp":1790789727048,"version":"4.1.0"},"reference-count":41,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2019,2,18]],"date-time":"2019-02-18T00:00:00Z","timestamp":1550448000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003125","name":"Suomen Kulttuurirahasto","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003125","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100011797","name":"Turun Yliopistollinen Keskussairaala","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100011797","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Intl Journal of Cancer"],"published-print":{"date-parts":[[2019,12]]},"abstract":"<jats:p>Neurofibromatosis type 1 (NF1) is a cancer predisposition syndrome with an incidence of 1:2,000. Patients with NF1 have an increased cancer risk and mortality, but there are no population\u2010based cohort studies specifically investigating the risk of childhood malignancies. We used the Finnish NF1 cohort to analyze the incidence, risk and prognosis of malignancies in NF1 patients &lt;20\u2009years of age. Persons born in 1987\u20132011 were included, and 524 persons were followed through the files of the Finnish Cancer Registry from birth up to age 20\u2009years. This amounted to 8,376 person years. Fifty\u2010three patients had cancer &lt;20\u2009years of age, yielding a standardized incidence ratio (SIR) of 35.6. The most frequent location of pediatric cancers was the central nervous system (CNS); there were 45 cases and the SIR was 115.7. Exclusion of 22 optic pathway gliomas (OPGs) gave an SIR of 59.1 for the CNS and 21.6 for all cancers. There were nine malignant peripheral nerve sheath tumors (MPNSTs); their cumulative risk was 2.7% by age 20. No cases of leukemia were observed. NF1 patients showed considerable excess mortality with a standardized mortality ratio (SMR) of 73.1. The survival of NF1 patients with CNS tumors other than OPGs did not differ from that of non\u2010NF1 controls (HR 0.64, 95% CI 0.23 to 1.76). In conclusion, brain tumors in childhood and MPNSTs in adolescence are malignancies of major concern in patients with NF1. The risk for myeloid malignancies may not be as high as suggested in the literature.<\/jats:p>","DOI":"10.1002\/ijc.32187","type":"journal-article","created":{"date-parts":[[2019,2,6]],"date-time":"2019-02-06T08:06:59Z","timestamp":1549440419000},"page":"2926-2932","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":51,"title":["Pediatric malignancies in neurofibromatosis type 1: A population\u2010based cohort study"],"prefix":"10.1002","volume":"145","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0990-1430","authenticated-orcid":false,"given":"Sirkku","family":"Peltonen","sequence":"first","affiliation":[{"name":"Department of Dermatology University of Turku Turku Finland"},{"name":"Department of Dermatology Turku University Hospital Turku Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roope A.","family":"Kallionp\u00e4\u00e4","sequence":"additional","affiliation":[{"name":"Institute of Biomedicine, University of Turku Turku Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matti","family":"Rantanen","sequence":"additional","affiliation":[{"name":"Finnish Cancer Registry \u2013 Institute for Statistical and Epidemiological Cancer Research Helsinki Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elina","family":"Uusitalo","sequence":"additional","affiliation":[{"name":"Institute of Biomedicine, University of Turku Turku Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P\u00e4ivi M.","family":"L\u00e4hteenm\u00e4ki","sequence":"additional","affiliation":[{"name":"Department of Pediatric and Adolescent Medicine Turku University Hospital and University of Turku Turku Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minna","family":"P\u00f6yh\u00f6nen","sequence":"additional","affiliation":[{"name":"Department of Clinical Genetics, HUSLAB Helsinki University Central Hospital Helsinki 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