Spinal tumours in neurofibromatosis type 1: an MRI study of frequency, multiplicity and variety.
In neurofibromatosis type 1 (NF1) spinal tumours cause neurological symptoms in about 2 % of patients. Among over 1400 patients with NF1 we saw symptomatic spinal tumours in 23 (1.6 %). MRI of the entire spinal canal was obtained in 54 patients aged 5-56 years with NF1. The number, site, morphology and signal characteristics of the spinal tumours were recorded and analysed. There were 24 patients with symptoms such as sensory impairment or paralysis; 30 patients had no neurological deficits. Of the 24 symptomatic patients, 23 (96 %) had spinal tumours, while we saw spinal tumours in 12 (40 %) of the 30 patients without neurological deficits. No spinal segment was preferred in symptomatic or asymptomatic patients. Most intraspinal extramedullary tumours were primarily extradural and intraforaminal. MRI showed intramedullary tumours in 3 patients (6 %), intraspinal extramedullary tumours in 18 (33 %) and intraforaminal tumours in 31 (57 %). Only neurological deficits in patients with NF1 should prompt further diagnostic clarification. In patients with neurological symptoms there may be a multiplicity of masses in the spinal canal, which can lead to difficulties in attaching symptoms to a certain tumour. In patients who do not satisfy the NIH criteria, it can be a helpful observation that spinal tumours in NF1 are primarily intraforaminal, extending into the spinal canal, while in NF2 they are mostly intraspinal intradural tumours.
Thakkar
S D
SD
Department of Neurology, Klinikum Nord Ochsenzoll, Langenhorner Chaussee 560, D-22419 Hamburg, Germany. sdthakkar@aol.com
Feigen
U
U
Mautner
V F
VF
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015174:Epidural Neoplasms / Q000175:diagnosis; D004824:Epidural Space / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000175:diagnosis*; D012680:Sensitivity and Specificity; D013115:Spinal Canal / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology; D013117:Spinal Cord Compression / Q000175:diagnosis; D013125:Spinal Neoplasms / Q000175:diagnosis*