NEUROSARCOMA IN A PATIENT WITH TYPE-1 NEU ROFIBROMATOSIS

BACKGROUND: Type 1 neurofibromatosis considerably increases the risk o f cancer development, particularly neurosarcoma. We report a case in a patient with chemosensitive metastatic neurosarcoma. CASE REPORT: A y oung female patient with familial type 1 neuro fibromatosis developed pleural metastasis of a neurosarcoma located on the arm. This tumor wa s initially highly sensitive to chemotherapy, but relapse occured. DIS CUSSION: Follow-up in the order members of the family was particulary difficult to organize. One sister developed cerebral astrocytoma. Neur osarcomas develop earlier in patients with type 1 neurofibromatosis, w orsening prognosis. We suggest a prospective and structured registrati on of such cases using a network of clinicians and pathologists in ord er to improve management schemes.