Localized crystal-storing histiocytosis of the posterior fossa

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

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

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

Bibliographical note

Funding Information:
Effort was partially supported by NIH grants P50 AG005136 (CDK) and P50 AG047366 (MEF), and the Nancy and Buster Alvord Endowment (CDK). We authors wish to thank Dr. Carolina Gil and Ms. Jocelyn Ricard (University of Minnesota, Minneapolis, MN) for providing important input on this work.

Publisher Copyright:
© 2018 Japanese Society of Neuropathology

Keywords

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

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