Could an osteoinductor result in degeneration of a neurofibroma in NF1?
Autor: | Jean-Paul, Steib, Steib, Jean-Paul, Julia, Bouchaïb, Bouchaïb, Julia, Axel, Walter, Walter, Axel, Sébastien, Schuller, Schuller, Sébastien, Yann Philippe, Charles, Charles, Philippe |
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Rok vydání: | 2010 |
Předmět: |
Male
medicine.medical_specialty Neurofibromatosis 1 medicine.medical_treatment Case Report Degeneration (medical) Young Adult Fatal Outcome Postoperative Complications Neurofibrosarcoma Biopsy medicine Humans Neurofibroma Orthopedics and Sports Medicine Neurofibromatosis Spinal Neoplasms medicine.diagnostic_test business.industry Laminectomy medicine.disease Surgery Bone Morphogenetic Proteins Disease Progression Neurosurgery Sarcoma business |
Zdroj: | European Spine Journal. 19:220-225 |
ISSN: | 1432-0932 0940-6719 |
DOI: | 10.1007/s00586-010-1416-8 |
Popis: | 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. |
Databáze: | OpenAIRE |
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