Second primary tumors in neurofibromatosis 1 patients treated for optic glioma: substantial risks after radiotherapy.
Authors
Sharif, SabaFerner, Rosalie
Birch, Jillian M
Gillespie, James E
Gattamaneni, Rao
Baser, Michael E
Evans, D Gareth R
Affiliation
Department of Clinical Genetics, St Mary's Hospital, Manchester, United Kingdom.Issue Date
2006-06-01
Metadata
Show full item recordAbstract
PURPOSE: Optic pathway gliomas (OPGs) are the most common CNS tumor in neurofibromatosis 1 (NF1) patients. We evaluated the long-term risk of second tumors in NF1-related OPGs after radiotherapy. PATIENTS AND METHODS: We reviewed 80 NF1 OPG patients from two NF1 clinics to evaluate the long-term risk of developing subsequent nervous system tumors, with or without radiotherapy. RESULTS: Fifty-eight patients were assessable for second tumors. Nine (50%) of 18 patients who received radiotherapy after their OPGs developed 12 second tumors in 308 person-years of follow-up after radiotherapy. Eight (20%) of 40 patients who were not treated with radiotherapy developed nine tumors in 721 person-years of follow-up after diagnosis of their OPGs. The relative risk of second nervous system tumor after radiotherapy was 3.04 (95% CI, 1.29 to 7.15). CONCLUSION: There is a significantly increased risk of second nervous system tumors in those NF1 patients who received radiotherapy for their OPGs, especially when treated in childhood. Thus radiotherapy should only be used if absolutely essential in children with NF1.Citation
Second primary tumors in neurofibromatosis 1 patients treated for optic glioma: substantial risks after radiotherapy. 2006, 24 (16):2570-5 J. Clin. Oncol.Journal
Journal of Clinical OncologyDOI
10.1200/JCO.2005.03.8349PubMed ID
16735710Type
ArticleLanguage
enISSN
1527-7755ae974a485f413a2113503eed53cd6c53
10.1200/JCO.2005.03.8349