title: The relationship between neurofibromatosis type 1, juvenile xanthogranuloma, and malignancy: A retrospective case-control study.
abstract: BACKGROUND
  Neurofibromatosis type 1 (NF-1) predisposes individuals to the development of benign and malignant tumors. The association of NF-1, juvenile xanthogranuloma (JXG), and juvenile myelomonocytic leukemia has been described in the literature. It is unclear whether JXG alone constitute a risk factor for leukemia or other malignancies in children with NF-1.
  
  
  OBJECTIVE
  To determine if there is an association between NF-1, JXG, and malignancy.
  
  
  METHODS
  We conducted a retrospective case-control study comparing children with NF-1 and malignancy (cases) with sex- and age-matched children with NF-1 without malignancy (controls).
  
  
  RESULTS
  We identified 739 patients with NF-1 over a 20-year period, 14 of whom also had a diagnosis of malignancy. These cases include 9 (64%) boys and 5 (36%) girls. JXG were found in 4/14 (28.5%) cases and 6/29 (21%) controls (odds ratio 1.5, 95% confidence interval 0.35-6.6, P = .56).
  
  
  CONCLUSIONS
  Retrospective design, small number of cases, and inconsistent documentation of clinical findings, including age at disappearance of JXG.
  
  
  CONCLUSIONS
  Juvenile xanthogranulomas do not appear to confer an increased risk for malignancy in children with NF-1.
authors.affiliation: Section of Pediatric Dermatology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Carmen
authors.initials: C
authors.lastname: Liy-Wong
authors.affiliation: Department of Pediatrics, Children's Hospital at London Health Science Centre, Western University, London.
authors.forename: Javed
authors.initials: J
authors.lastname: Mohammed
authors.affiliation: Department of Family Medicine, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Amanda
authors.initials: A
authors.lastname: Carleton
authors.affiliation: Section of Pediatric Dermatology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Elena
authors.initials: E
authors.lastname: Pope
authors.affiliation: Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Patricia
authors.initials: P
authors.lastname: Parkin
authors.affiliation: Section of Pediatric Dermatology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: Irene.lara-corrales@sickkids.ca.
authors.forename: Irene
authors.initials: I
authors.lastname: Lara-Corrales
mesh_terms: D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D009369:Neoplasms / Q000453:epidemiology / Q000209:etiology*; D009456:Neurofibromatosis 1 / Q000150:complications*; D012189:Retrospective Studies; D018570:Risk Assessment; D014972:Xanthogranuloma, Juvenile / Q000150:complications*
keywords: juvenile xanthogranuloma; leukemia; malignancy; malignant tumors; neurofibromatosis type 1