title: [Neurofibrosarcomas in neurofibromatosis 1].
abstract: Between 1983 and 1987 three patients (mean age 31 years) with neurofibrosarcoma were seen among 22 with von Recklinghausen neurofibromatosis (NF-1) at the Department of Neurology, University of Berne, Switzerland. There was an average interval of 14 months between onset of symptoms and treatment. The first presenting sign was a rapidly enlarging mass or unusual persistent pain. The occurrence of either sign, in the known presence of NF-1, should lead to immediate neuroradiological investigation. The mean survival time of the three patients was 37 months. Only early diagnosis provides any chance of curative treatment.
authors.affiliation: Neurologische Universitätsklinik, Inselspital, Bern.
authors.forename: J P
authors.initials: JP
authors.lastname: Sieb
authors.forename: H
authors.initials: H
authors.lastname: Mattle
authors.forename: M
authors.initials: M
authors.lastname: Pirovino
mesh_terms: D000328:Adult; D001859:Bone Neoplasms / Q000175:diagnosis / Q000473:pathology; D005260:Female; D018219:Histiocytoma, Benign Fibrous / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D007085:Ilium; D008297:Male; D008475:Median Nerve; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000473:pathology; D009455:Neurofibroma / Q000175:diagnosis* / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000473:pathology; D010386:Pelvic Neoplasms / Q000175:diagnosis / Q000473:pathology; D013997:Time Factors
vernacular_title: Neurofibrosarkome bei Neurofibromatose 1.