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Acta Neuropathologica

, Volume 110, Issue 3, pp 306–311 | Cite as

Pilocytic astrocytoma presenting as primary diffuse leptomeningeal gliomatosis: report of a unique case and review of the literature

  • Georg Bohner
  • Florian Masuhr
  • Roland Distl
  • Juri Katchanov
  • Randolf Klingebiel
  • Rolf Zschenderlein
  • Andreas von Deimling
  • Frank K. H. van Landeghem
Case Report

Abstract

We describe a 25-year-old male patient with primary diffuse leptomeningeal gliomatosis (PDLG) presenting with gait ataxia, positive Lhermitte’s sign, double vision, and right abducens nerve palsy. Spinal magnetic resonance imaging showed extended intradural, extramedullary, contrast-enhancing masses with compression of the myelon. Spinal leptomeningeal biopsy revealed a pilocytic astrocytoma WHO grade I. Despite chemotherapy with vincristin and carboplatin, the patient died 2 months after admission. A thorough autopsy showed no evidence for primary neoplasms in brain, spine and optic nerve. Sequence analysis of tumor protein 53 gene (TP53) revealed a missense mutation in exon 5, and expression of phosphatase and tensin homolog (mutated in multiple advanced cancers 1) (PTEN) protein was not detected, which may have contributed to astrocytoma development. To our knowledge, this is the first definitive case of pilocytic astrocytoma presenting as PDLG.

Keywords

Pilocytic astrocytoma Primary diffuse leptomeningeal gliomatosis TP53 

Notes

Acknowledgements

We thank Karl Riabowol for critically reading and discussing our manuscript. We thank Petra Matylewski and Ulrike Lass for excellent technical assistance.

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Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • Georg Bohner
    • 1
  • Florian Masuhr
    • 2
  • Roland Distl
    • 3
  • Juri Katchanov
    • 2
  • Randolf Klingebiel
    • 1
  • Rolf Zschenderlein
    • 2
  • Andreas von Deimling
    • 3
  • Frank K. H. van Landeghem
    • 3
  1. 1.Department of Radiology, Neuroradiology SectionCharité Medical CenterBerlinGermany
  2. 2.Department of NeurologyCharité Medical CenterBerlinGermany
  3. 3.Institute of NeuropathologyCharité Medical CenterBerlinGermany

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