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Published in: Child's Nervous System 10/2007

01-10-2007 | Case Report

Multiple pilocytic astrocytomas of the cerebellum in a 17-year-old patient with neurofibromatosis type I

Authors: Ian F. Dunn, Pankaj K. Agarwalla, Alexander M. Papanastassiou, William E. Butler, Edward R. Smith

Published in: Child's Nervous System | Issue 10/2007

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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.

Literature
  1. Bilaniuk LT, Molloy PT, Zimmerman RA, Phillips PC, Vaughan SN, Liu GT, Sutton LN, Needle M (1997) Neurofibromatosis type 1: brain stem tumours. Neuroradiology 39:642–653PubMedView Article
  2. Gilles FH, Leviton A, Hedley-Whyte ET, Sobel E, Tavare CJ, Sobel RS, Rorke LB (1992) Childhood brain tumors that occupy more than one compartment at presentation. Multiple compartment tumors. J Neurooncol 14:45–56PubMedView Article
  3. Guillamo JS, Creange A, Kalifa C, Grill J, Rodriguez D, Doz F, Barbarot S, Zerah M, Sanson M, Bastuji-Garin S, Wolkenstein P (2003) Prognostic factors of CNS tumours in neurofibromatosis 1 (NF1): a retrospective study of 104 patients. Brain 126:152–160PubMedView Article
  4. Ilgren EB, Kinnier-Wilson LM, Stiller CA (1985) Gliomas in neurofibromatosis: a series of 89 cases with evidence for enhanced malignancy in associated cerebellar astrocytomas. Pathol Annu 20(Pt 1):331–358PubMed
  5. Matsumoto T, Uekusa T, Abe H, Fukuda Y, Mizutani Y, Oikawa S, Doi K, Imai H, Sato T (1989) Multicentric astrocytomas of the optic chiasm, brain stem and spinal cord. Acta Pathol Jpn 39:664–669PubMed
  6. Mukai K, Kitamura K, Asano N, Ohshima T, Hondo H, Matsumoto K (1989) Multifocal gliomas in cerebral hemisphere associated with von Recklinghausen’s disease: case report. No Shinkei Geka 17:197–202PubMed
  7. Riccardi VM (1991) Neurofibromatosis: past, present, and future. N Engl J Med 324(18)1283–1285View Article
  8. Sakaida H, Hanakita J, Suwa H, Nagayasu S, Nishi S, Ohta F (1992) Two cases of von Recklinghausen’s disease with multiple brain and spinal tumors. No Shinkei Geka 20:51–56PubMed
  9. Senaratna S, Hanieh A, Manson J, Toogood I (2001) Multiple cystic brain lesions in a patient with pilocytic astrocytoma. J Clin Neurosci 8:363–366PubMedView Article
  10. Sorensen SA, Mulvihill JJ, Nielsen A (1986) Long-term follow-up of von Recklinghausen neurofibromatosis. Survival and malignant neoplasms. N Engl J Med 314:1010–1015PubMedView Article
Metadata
Title
Multiple pilocytic astrocytomas of the cerebellum in a 17-year-old patient with neurofibromatosis type I
Authors
Ian F. Dunn
Pankaj K. Agarwalla
Alexander M. Papanastassiou
William E. Butler
Edward R. Smith
Publication date
01-10-2007
Publisher
Springer-Verlag
Published in
Child's Nervous System / Issue 10/2007
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-007-0343-4

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