Childhood neurofibromatosis: risk factors for malignant disease |
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| Authors: | M Schneider A C Obringer E Zackai A T Meadows |
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| Institution: | 1. Institute of Biostructure and Bioimaging (CNR) c/o Molecular Biotechnologies Center, Via Nizza 52, 10126, Torino, Italy;2. Molecular Imaging Center, University of Torino, Via Nizza 52, 10126 Torino, Italy;3. Department of Molecular Biotechnology and Health Sciences, University of Torino, Via Nizza 52, 10126 Torino, Italy;4. Dept. of Computer Science, University College London, Gower Street, London WC1E 6BT, United Kingdom;1. Division of Mathematical oncology, City of Hope National Medical Center, CA, USA;2. Department of Radiology and Imaging, Fortis Memorial Research Institute, Gurgaon, India;3. Department of Biostatistics and Health Informatics, SGPGIMS, Lucknow, India;4. Department of Neurosurgery, Fortis Memorial Research Institute, Gurgaon, India;5. SRL Diagnostics, Fortis Memorial Research Institute, Gurgaon, India;6. Philips Health System, Philips India Limited, Gurgaon, India |
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| Abstract: | Forty-five children with both neurofibromatosis and malignant tumors were compared with a pediatric population with neurofibromatosis without tumors to ascertain if any of the clinical manifestations of this disorder were associated with either tumor development or histology. No such factors emerged except that, in most of the optic glioma patients, the neurofibromatosis mutation was paternally inherited. Concordance of histologic tumor types was seen when malignancies occurred in affected family members, similar to that noted in the literature. Although cancer is independent of the other manifestations of neurofibromatosis and these manifestations may vary among family members, it appears that malignant tumors are often concordant when they occur within a family. |
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