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.
Bibliographical noteFunding 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.
© 2018 Japanese Society of Neuropathology
- central nervous system
- crystal-storing histiocytosis
- electron microscopy
- intracellular crystals
- lymphoplasmacytic neoplasm