[Neurosarcoma associated with neurofibromatosis 1. Apropos of a case and review of the literature].
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.
Ligneau
B
B
Service de Radiothérapie-Oncologie, Centre Hospitalier Lyon-Sud, Pierre-Bénite.
Freyer
G
G
Giraud
S
S
Isaac
S
S
Bombaron
P
P
Espinouse
D
D
Plauchu
H
H
Fischer
G
G
Gérard
J P
JP
Lenoir
G
G
Trillet-Lenoir
V
V
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*