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Subsequent neoplasms after a primary tumor in individuals with neurofibromatosis type 1

Journal of Clinical Oncology Sep 27, 2019

Bhatia S, Chen Y, Wong FL, et al. - Researchers investigated the risk of subsequent neoplasms (SNs) in children with pathogenic neurofibromatosis type 1 (NF1) variants exposed to radiation and/or alkylator chemotherapy, in order to fill the existing knowledge gaps. From the Childhood Cancer Survivor Study, they compared 167 NF1-affected vs 1,541 non–NF1-affected 5-year childhood cancer survivors with respect to the SN risk, and 176 nonoverlapping NF1-affected people with primary tumors exposed to radiation and/or chemotherapy were selected from the University of Alabama at Birmingham and Children’s Hospital of Philadelphia. They found an increased risk of SN in children with NF1 who developed a primary tumor vs non-NF1 childhood cancer survivors. An increased risk of SNs was reported in correlation with therapeutic radiation, but not alkylating agents, in NF1-affected children with a primary tumor.
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