Population-based study of impact of neurofibromatosis 1 on cancer survival in Denmark

Population-based evidence on cancer survival in individuals with neurofibromatosis 1 (NF1) is scarce. A population-based study in Denmark investigated survival following a first neoplasm in individuals with NF1 compared to that of the general Danish population. In the Danish Cancer Registry, the authors identified 428 individuals with NF1 and 392,885 without NF1 diagnosed with a first neoplasm between 1977 and 2022. Among children (aged 1-19 years), NF1 was overall associated with better 5-year survival (87.3%; 95% CI 81.1-93.5) compared to the general population (78.7%; 95% CI 77.9-79.6), due to better survival following central nervous system neoplasms. In adults, 5-year survival was lower in NF1 patients (57.0%; 95% CI 51.3-62.6) compared to general population (69.2%; 69.0-69.3). NF1 aults more often presented wirh stage IV breast (17.2% versus 6.3%) and gastrointestinal cancers (52.5% versus 37.7%). Five-year neoplastic mortality was higher in NF1 adults (39.4% versus 27.7%). The authors conclude that individuals with NF1, particularly adults, have poorer cancer survival than the general population. Differences in cancer type and stage at diagnosis contribute to but do not fully explain the excess mortality (graphical abstract).