Localized crystal-storing histiocytosis of the posterior fossa

Margaret E. Flanagan, Christopher Dirk Keene, David N. Louis, Gordana Juric-Sekhar*

*Corresponding author for this work

Research output: Contribution to journalArticle

Abstract

Crystal-storing histiocytosis (CSH) is an uncommon histiocytic proliferation reported to involve diverse organs and tissues, but involvement of the central nervous system (CNS) is rare. In most cases CSH is identified in association with underlying lymphoproliferative, plasma cell diseases or rarely with various inflammatory or infectious conditions. CSH is characterized by the cytoplasmic accumulation of crystalline material in histiocytes, most commonly of kappa immunoglobulin light chain. We report a unique case of localized CSH involving the left cerebellum and caudal brain stem in a young man with a history of gout but without known lymphoproliferative or plasma cell disorders. Awareness of this entity is important diagnostically, but also to ensure appropriate management and follow-up, particularly in the absence of apparent underlying malignancy.

Original languageEnglish (US)
Pages (from-to)529-534
Number of pages6
JournalNeuropathology
Volume38
Issue number5
DOIs
StatePublished - Oct 2018

Keywords

  • central nervous system
  • crystal-storing histiocytosis
  • electron microscopy
  • intracellular crystals
  • lymphoplasmacytic neoplasm

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology

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