Neurofibromatosis type 1: brain stem tumours.
Publication/Presentation Date
9-1-1997
Abstract
We describe the clinical and imaging findings of brain stem tumours in patients with neurofibromatosis type 1 (NF1). The NF1 patients imaged between January 1984 and January 1996 were reviewed and 25 patients were identified with a brain stem tumour. Clinical, radiographical and pathological results were obtained by review of records and images. Brain stem tumour identification occurred much later than the clinical diagnosis of NF1. Medullary enlargement was most frequent (68%), followed by pontine (52%) and midbrain enlargement (44%). Patients were further subdivided into those with diffuse (12 patients) and those with focal (13 patients) tumours. Treatment for hydrocephalus was required in 67% of the first group and only 15% of the second group. Surgery was performed in four patients and revealed fibrillary astrocytomas, one of which progressed to an anaplastic astrocytoma. In 40% of patients both brain stem and optic pathway tumours were present. The biological behaviour of brain stem tumours in NF1 is unknown. Diffuse tumours in the patients with NF1 appear to have a much more favourable prognosis than patients with similar tumours without neurofibromatosis type 1.
Volume
39
Issue
9
First Page
642
Last Page
653
ISSN
0028-3940
Published In/Presented At
Bilaniuk, L. T., Molloy, P. T., Zimmerman, R. A., Phillips, P. C., Vaughan, S. N., Liu, G. T., Sutton, L. N., & Needle, M. (1997). Neurofibromatosis type 1: brain stem tumours. Neuroradiology, 39(9), 642–653. https://doi.org/10.1007/s002340050484
Disciplines
Medicine and Health Sciences | Pediatrics
PubMedID
9335063
Department(s)
Department of Pediatrics
Document Type
Article