title: Could an osteoinductor result in degeneration of a neurofibroma in NF1?
abstract: We report a case of fatal evolution of neurofibromatosis in a young boy. A laminectomy was performed when he was 9 years old. A secondary hyperkyphosis led to many surgeries resulting in recurrent malunions. When he was 23 years old, a breakage of his rods was treated by a new instrumentation and a T12-L1 interbody cage fitted with rh-BMP. Five months later, he developed a huge posterior tumour on his back. The biopsy diagnosed a neurofibrosarcoma. The growth of the tumour was extremely rapid. He died after several months from a septic shock. NF1 is characterised by neurofibromas that have a possibility of malign degeneration and conversion to a sarcoma. However, the chronology, rapidity of evolution and the exceptional volume of the tumour made us wonder whether the BMP had a part of responsibility as osteoinductor in the malignant degeneration, in this particular case, of neurofibromatosis. It seemed important to point out this case to the medical community.
authors.affiliation: Service de Chirurgie du Rachis, Hôpitaux Universitaires de Strasbourg, Pavillon Chirurgical B, BP 426, 67091 Strasbourg Cedex, France. jean-paul.steib@chru-strasbourg.fr
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Steib
authors.forename: Steib
authors.initials: S
authors.lastname: Jean-Paul
authors.forename: Julia
authors.initials: J
authors.lastname: Bouchaïb
authors.forename: Bouchaïb
authors.initials: B
authors.lastname: Julia
authors.forename: Axel
authors.initials: A
authors.lastname: Walter
authors.forename: Walter
authors.initials: W
authors.lastname: Axel
authors.forename: Sébastien
authors.initials: S
authors.lastname: Schuller
authors.forename: Schuller
authors.initials: S
authors.lastname: Sébastien
authors.forename: Yann Philippe
authors.initials: YP
authors.lastname: Charles
authors.forename: Charles
authors.initials: C
authors.lastname: Philippe
mesh_terms: D019485:Bone Morphogenetic Proteins / Q000009:adverse effects*; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D009455:Neurofibroma / Q000473:pathology / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000473:pathology / Q000601:surgery; D018319:Neurofibrosarcoma / Q000139:chemically induced* / Q000473:pathology* / Q000503:physiopathology; D011183:Postoperative Complications / Q000188:drug therapy*; D013125:Spinal Neoplasms / Q000139:chemically induced* / Q000473:pathology* / Q000503:physiopathology; D055815:Young Adult
chemical_list: D019485:Bone Morphogenetic Proteins
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