title: [Neurosarcoma associated with neurofibromatosis 1. Apropos of a case and review of the literature].
abstract: BACKGROUND
  Type 1 neurofibromatosis considerably increases the risk of cancer development, particularly neurosarcoma. We report a case in a patient with chemosensitive metastatic neurosarcoma.
  
  
  METHODS
  A young female patient with familial type 1 neurofibromatosis developed pleural metastasis of a neurosarcoma located on the arm. This tumor was initially highly sensitive to chemotherapy, but relapse occurred.
  
  
  CONCLUSIONS
  Follow-up in the order members of the family was particularly difficult to organize. One sister developed cerebral astrocytoma. Neurosarcomas develop earlier in patients with type 1 neurofibromatosis, worsening prognosis. We suggest a prospective and structured registration of such cases using a network of clinicians and pathologists in order to improve management schemes.
authors.affiliation: Service de Radiothérapie-Oncologie, Centre Hospitalier Lyon-Sud, Pierre-Bénite.
authors.forename: B
authors.initials: B
authors.lastname: Ligneau
authors.forename: G
authors.initials: G
authors.lastname: Freyer
authors.forename: S
authors.initials: S
authors.lastname: Giraud
authors.forename: S
authors.initials: S
authors.lastname: Isaac
authors.forename: P
authors.initials: P
authors.lastname: Bombaron
authors.forename: D
authors.initials: D
authors.lastname: Espinouse
authors.forename: H
authors.initials: H
authors.lastname: Plauchu
authors.forename: G
authors.initials: G
authors.lastname: Fischer
authors.forename: J P
authors.initials: JP
authors.lastname: Gérard
authors.forename: G
authors.initials: G
authors.lastname: Lenoir
authors.forename: V
authors.initials: V
authors.lastname: Trillet-Lenoir
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D018319:Neurofibrosarcoma / Q000473:pathology* / Q000556:secondary / Q000628:therapy; D012983:Soft Tissue Neoplasms / Q000473:pathology*