Objective: The need for radiologic surveillance of spinal tumors in children with neurofibromatosis 1 is controversial and unresolved. We aimed to determine the incidence of spinal tumors in asymptomatic patients, describe the imaging findings, and evaluate the clinical usefulness of a prospective MR imaging surveillance program of the spine in children with neurofibromatosis 1.
Subjects and methods: Of 62 children consecutively seen in a neurofibromatosis 1 clinic, 53 (85.5%) were recruited for MR imaging of the whole spine. All children satisfied the clinical criteria for diagnosis of neurofibromatosis 1. Imaging findings, clinical signs and symptoms, and subsequent clinical management were reviewed.
Results: The patients were 35 boys and 18 girls (age range, 11 months-18 years; mean age, 9.6 years), all of whom were asymptomatic, with no remarkable neurologic signs. Seven children (13.2%) had spinal neurofibromas: four had solitary neurofibromas (two dumbbell, one intradural, and one paraspinal tumor) and three had plexiform neurofibromas of the sacral plexus and thoracic and lumbar nerve sheaths. The incidences of scoliosis, localized cutaneous neurofibromas, and massive soft-tissue neurofibromas were 71.4%, 71.4%, and 28.6%, respectively, in the group with spinal neurofibromas (n = 7), and 30.4%, 39.1%, and 8.7%, respectively, in the group without spinal neurofibromas (n = 46). Patient clinical outcome was affected in only one patient (1.9%) in whom a solitary neurofibroma was resected. Follow-up imaging in 10 patients (mean period, 29 months) showed no evidence of tumor occurrence, progression, or recurrence.
Conclusion: Although benign spinal neurofibromas are not uncommon in asymptomatic children with neurofibromatosis 1, the clinical usefulness of spine surveillance with MR imaging is limited in these children, making its effectiveness questionable.