Benign brain stem lesions in pediatric patients with neurofibromatosis: case reports.
The symptoms and clinical courses of 4 patients with neurofibromatosis and lesions of the brain stem identifiable on computed tomographic and/or magnetic resonance imaging scans are described. Two patients underwent biopsy and both had low-grade astrocytomas with no evidence of anaplasia. Both received radiation and chemotherapy. The other 2 patients have been monitored without biopsy or treatment. Three patients are alive and clinically stable, having been followed up for an average of 4 years; neuroimaging studies have shown no change in their tumors. The fourth patient died of a supratentorial primitive neuroectodermal tumor. Imaging studies had shown no change in his brain stem lesion, which at autopsy was found to be a focal collection of fibrillary astrocytes. These data suggest that some patients with brain stem lesions and neurofibromatosis may have a prognosis distinctly different from that of the typical patient with a brain stem glioma. We recommend caution against aggressive operative and adjuvant therapy for brain stem lesions in patients with neurofibromatosis, unless progression of the lesion is documented clinically and/or by imaging.
Raffel
C
C
Divisions of Neurosurgery, Children's Hospital of Los Angeles, California.
McComb
J G
JG
Bodner
S
S
Gilles
F E
FE
D000293:Adolescent; D001254:Astrocytoma / Q000150:complications* / Q000175:diagnosis / Q000000981:diagnostic imaging; D001932:Brain Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000000981:diagnostic imaging; D001933:Brain Stem / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009456:Neurofibromatosis 1 / Q000150:complications*; D014057:Tomography, X-Ray Computed