ArticleInternational journal of cancer2019
Pediatric malignancies in neurofibromatosis type 1: A population-based cohort study.
Article in International journal of cancer, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers.
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Who cites it
33 citing papers in PubMed.
- Hospital contacts before childhood cancer and the risk of early mortality: a Danish and Swedish population-based cohort study.British journal of cancer · 2026Article
- Retrospective Analysis of T2-Hyperintense Lesions in Children With Neurofibromatosis Type 1.Annals of the Child Neurology Society · 2026Article
- Circulating Tumor DNA in Neurofibromatosis Type 1: Translating Molecular Discovery into Clinical Surveillance.Diagnostics (Basel, Switzerland) · 2026Review
- Neurofibromatosis type 1 with concomitant Peutz-Jeghers syndrome in a child: a case report.BMC pediatrics · 2026Article
- Article
- Diagnosis, treatment management, and challenges in neurofibromatosis type 1: a case report with mixed features.Annals of medicine and surgery (2012) · 2026Article
- Neoplasm risk in individuals with neurofibromatosis 1 - a Danish nationwide cohort study with long-term follow-up.British journal of cancer · 2026Article
- Frequency and clinical features of germline pathogenic variants in sarcoma: a case-control study.Journal of the National Cancer Institute · 2026Article
- Mortality after cancer diagnosis among children with congenital heart disease in Denmark and Sweden.Journal of the National Cancer Institute · 2025Article
- Pregnancy and the Risk for Cancer in Neurofibromatosis 1.Genes, chromosomes & cancer · 2025Article
- Article
- PET Imaging of Neurofibromatosis Type 1 with a Fluorine-18 Labeled Tryptophan Radiotracer.Pharmaceuticals (Basel, Switzerland) · 2024Article
- Perinatal folate levels do not influence tumor latency or multiplicity in a model of NF1 associated plexiform-like neurofibromas.BMC research notes · 2023Article
- Neurocutaneous Syndromes, Perinatal Factors, and the Risk of Childhood Cancer in Sweden.JAMA network open · 2023Article
- Loss of Nf1 and Ink4a/Arf Are Associated with Sex-Dependent Growth Differences in a Mouse Model of Embryonal Rhabdomyosarcoma.Current issues in molecular biology · 2023Article
- ERN GENTURIS tumour surveillance guidelines for individuals with neurofibromatosis type 1.EClinicalMedicine · 2023Review
- Nervous system (NS) Tumors in Cancer Predisposition Syndromes.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2022Review
- Systematic Review of Pediatric Brain Tumors in Neurofibromatosis Type 1: Status of Gene Therapy.Cureus · 2022Review
- [Progress in diagnosis and treatment of neurofibromatosis in children].Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery · 2022Review
- Current concepts of neurofibromatosis type 1: pathophysiology and treatment.Archives of craniofacial surgery · 2022Article
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8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
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-based 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 <20 years of age. Persons born in 1987-2011 were included, and 524 persons were followed through the files of the Finnish Cancer Registry from birth up to age 20 years. This amounted to 8,376 person years. Fifty-three patients had cancer <20 years 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-NF1 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.
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