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Second Primary Tumors in Neurofibromatosis 1 Patients Treated for Optic Glioma: Substantial Risks After Radiotherapy

Saba Sharif, Rosalie Ferner, Jillian M. Birch, James E. Gillespie, H. Rao Gattamaneni, Michael E. Baser, D. Gareth R. Evans

From the Department of Clinical Genetics, St Mary's Hospital; Cancer Research UK Paediatric and Familial Cancer Research Group, Royal Manchester Children's Hospital; Department of Clinical Radiology, Manchester Royal Infirmary; Christie Hospital, Manchester; Department of Clinical Neurosciences, Guy's Kings and St Thomas' School of Medicine, Guy's Hospital, London, United Kingdom; and private practice, Los Angeles, CA

Address reprint requests to D. Gareth R. Evans, MD, FRCP, Department of Clinical Genetics, St Mary's Hospital (SM2), Hathersage Rd, Manchester M13 OJH, United Kingdom; e-mail: Gareth.evans{at}cmmc.nhs.uk

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.

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

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