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Primary leptomeningeal low-grade Schwann cell neoplasm
  1. Wilson Vallat1,
  2. Siby Antony2,
  3. Sandy Patel3 and
  4. Catriona McLean4,5
  1. 1Neurology, Lyell McEwin Hospital, Adelaide, South Australia, Australia
  2. 2Medicine, Lyell McEwin Hospital, Adelaide, South Australia, Australia
  3. 3Department of Radiology, Royal Adelaide Hospital, Adelaide, South Australia, Australia
  4. 4Anatomical Pathology, Alfred Health, Prahan, Victoria, Australia
  5. 5Victorian Brain Bank, The Florey Institute of Neuroscience and Mental Health, Melbourne, Victoria, Australia
  1. Correspondence to Dr Wilson Vallat; wilson.vallat{at}sa.gov.au

Abstract

We discuss an extremely rare case of low-grade Schwann cell leptomeningeal neoplasm with no evident intradural primary, presenting with rapid neurological decline leading to death reflecting the aggressive biological behaviour of this entity despite its low-grade morphology. Notwithstanding extensive investigations, the diagnosis was only established on autopsy as clinical presentation is non-specific making diagnosis challenging. This condition could be considered in patients presenting with leptomeningeal disease if initial workup of more common causes is non-revealing.

  • neurooncology
  • neuroimaging
  • CNS cancer
  • pathology

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Footnotes

  • Contributors All authors listed have contributed to the design and drafting of this work. WV: case vignette and literature review. SA: case vignette and literature review. SP: case vignette and neuroradiology. CM: case vignette, literature review, pathology and critical revision.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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