Cervical neurofibromas in children with NF-1

Abstract

Background. Children with neurofibromatosis type 1 (NF1) are at increased risk of developing plexiform neurofibroma throughout the body, including the cervical soft tissues. However, the incidence of cervical soft tissue tumors and the value of screening MR for children with NF1 are not known. Purpose. The purposes of this study were to determine the incidence and clinical significance of cervical tumors seen on MR imaging in children with NF1. Materials and methods. A retrospective review of the brain and orbit MR with cervical images obtained on 95 children who meet the NIH consensus criteria for NF1 and who are followed at our neurofibromatosis clinic was carried out. Results. Cervical tumors were found on MR imaging in 21 of 95 (22 %) children. Of 21 children with cervical tumors, 14 children were determined to be surgical candidates. In nine children, MR imaging altered the clinical management by demonstrating tumors for which surgery was indicated, but the tumors were not suspected prior to MR imaging. Conclusion. Cervical tumors are commonly seen in children with NF1. MR imaging may demonstrate a significant number of tumors that require surgery, but were not suspected prior to MR imaging.