title: Bone sarcomas arising in patients with neurofibromatosis type 1.
abstract: We identified eight patients of 2900 with a primary malignant bone tumour who had coexisting neurofibromatosis type 1. This was a much higher incidence than would be expected by chance. The patients had a mean age of 22.4 years (9 to 54): five were male. Two patients subsequently developed a second bone sarcoma, one of which was radiation induced. Four of the primary tumours were osteosarcomas, four were spindle-cell sarcomas and one a Ewing's sarcoma. All the patients were treated with chemotherapy and surgery: six of the eight appear to be cured. This study suggests a possible relationship between neurofibromatosis type 1 and the development of a bone sarcoma, the increased risk being estimated at eight times that of the normal population. We recommend that further research into this possible link should be considered.
authors.affiliation: Royal Orthopaedic Hospital, Bristol Road South, Northfield, Birmingham B31 2AP, UK.
authors.forename: M
authors.initials: M
authors.lastname: Chowdhry
authors.forename: C
authors.initials: C
authors.lastname: Hughes
authors.forename: R J
authors.initials: RJ
authors.lastname: Grimer
authors.forename: V
authors.initials: V
authors.lastname: Sumathi
authors.forename: S
authors.initials: S
authors.lastname: Wilson
authors.forename: L
authors.initials: L
authors.lastname: Jeys
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000473:pathology* / Q000628:therapy; D002648:Child; D004307:Dose-Response Relationship, Radiation; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000473:pathology* / Q000628:therapy; D009381:Neoplasms, Radiation-Induced / Q000473:pathology / Q000628:therapy; D009456:Neurofibromatosis 1 / Q000473:pathology* / Q000628:therapy; D012516:Osteosarcoma / Q000473:pathology* / Q000628:therapy; D012189:Retrospective Studies; D012983:Soft Tissue Neoplasms / Q000473:pathology* / Q000628:therapy; D055815:Young Adult