Observed and Expected Numbers of Cancer Cases and SIRs With 95% CIs Among Finnish Patients With NF1 from 1987 to 2012, by Cancer Type

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... country-wide Finnish Cancer Registry 23 was done automatically using patient personal identity codes as a key. We refer to cancers with ICD-10 codes C47, C48-49, and C70-72-cancers of autonomic nervous system, soft tissue, brain, and CNS-as NF1-specific cancers. Our research protocol also included an exhaustive morphologic subtype analysis using International Classification of Diseases for Oncology, 3rd Edition (ICD-O-3). MPNST was identified with ICD-O-3 morphology codes 9540/3 and 9560/3. Dates and causes of death were obtained from Statistics Finland and from the national Population Register Centre. For cancer deaths, we used the definitions of the Finnish Cancer Registry. 24 ...

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... NF1-specific cancers were not restricted to young age (Appendix Fig A1). SIR for MPNST was 2,056, with 58 observed cases in the entire cohort (Table 3). The cumulative risks for MPNST by age 30 years, age 50 years, and lifetime were estimated to be 8.5%, 12.3%, and 15.8%, respectively (Table 1). ...

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... cancers. With the exclusion of NF1-specific cancers, that is, nervous system and soft tissue malignancies, SIR for cancer remained elevated, for both men (1.96) and women (1.98; Table 3). Cancers with increased incidence were mainly breast and gastrointestinal cancers. ...

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... excess incidence of breast cancer was highest in young women. Specifically, SIR for breast cancer was 11.1 (95% CI, 5.56 to 19.5; P , .001) in Table 3). However, the incidence of leukemias or lymphomas was not increased, and subset analysis identified no cases of juvenile myelomonocytic leukemia. ...