Multiple pilocytic astrocytomas of the cerebellum in a 17-year-old patient with neurofibromatosis type I |
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Authors: | Ian F Dunn Pankaj K Agarwalla Alexander M Papanastassiou William E Butler Edward R Smith |
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Affiliation: | (1) Department of Neurosurgery, Children’s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USA;(2) Department of Neurosurgery, Massachusetts General Hospital, Boston, MA, USA |
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Abstract: | Objective Approximately 10% of patients with neurofibromatosis I (NFI) patients will have central nervous system (CNS) tumors. The most
common of these are hypothalamic–optic gliomas, followed by brainstem and cerebellar pilocytic astrocytomas. While isolated
pilocytic astrocytomas in NFI are well described, the appearance of multiple pilocytic astrocytomas in an individual patient
is less common. The most frequent combination in NFI patients with more than one pilocytic astrocytoma is optic tract/hypothalamic
and brainstem. Other combinations are exceedingly rare; multiple pilocytic astrocytomas have only been reported once in the
cerebral hemispheres in a patient with NFI. This report presents the first documented case, to our knowledge, of multiple
pilocytic astrocytomas in the cerebellum of a patient with NF1.
Methods Case report.
Conclusion The finding of multiple cerebellar pilocytic astrocytomas in a patient with NF1 is important because it expands the spectrum
of presentations for patients with NF1 and also highlights specific diagnostic and therapeutic challenges faced by the treating
physicians. The genetic and molecular basis of NF1 is reviewed. Strategies of diagnosis and treatment outlined here are relevant
to both patients with NF1 and all patients with multiple posterior fossa tumors. |
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Keywords: | Neurofibromatosis I Multiple pilocytic astrocytomas Brain tumor Pediatric |
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