title: Familial neuroblastoma: report of a kindred with later age at diagnosis.
abstract: OBJECTIVE
  To describe the clinical and biologic features of neuroblastoma (NB) in two siblings and their maternal second cousin.
  
  
  METHODS
  NB was diagnosed in the siblings at 2 1/2 (patient 2) and 5 (patient 3) years of age. NB was diagnosed in their maternal second cousin (patient 1) when she was 7 years old. Standard clinical and biological data, tumor karyotype, and tumor allelotype at select loci were obtained.
  
  
  RESULTS
  Patient 1 had International Neuroblastoma Staging System (INSS) stage 4 NB and unfavorable histology but no evidence of MYCN amplification; she died from complications of autologous bone marrow transplantation in second remission. Patient 2 had INSS stage 4 NB with unfavorable histology but no MYCN amplification; her disease recurred 39 months after completing therapy. Patient 3 had INSS stage 1 NB with favorable biologic features; he was treated with surgical excision and remains free of disease.
  
  
  CONCLUSIONS
  Familial NB may occur at a later age than predicted by the tumor suppressor gene model of inherited cancer. This report further emphasizes the clinical and biological heterogeneity of familial NB.
authors.affiliation: Department of Pediatrics, University of Manitoba, Winnipeg, Canada.
authors.forename: E G
authors.initials: EG
authors.lastname: Lemire
authors.forename: B N
authors.initials: BN
authors.lastname: Chodirker
authors.forename: G J
authors.initials: GJ
authors.lastname: Williams
authors.forename: L E
authors.initials: LE
authors.lastname: Seargeant
authors.forename: S J
authors.initials: SJ
authors.lastname: Israels
authors.forename: S M
authors.initials: SM
authors.lastname: Phillips
authors.forename: J A
authors.initials: JA
authors.lastname: de Nanassy
authors.forename: J M
authors.initials: JM
authors.lastname: Maris
authors.forename: R A
authors.initials: RA
authors.lastname: Yanofsky
mesh_terms: D017668:Age of Onset; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D016259:Genes, myc; D005819:Genetic Markers; D006801:Humans; D008297:Male; D009447:Neuroblastoma / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology; D010375:Pedigree
chemical_list: D005819:Genetic Markers