Abstract
Background
Crystal-storing histiocytosis (CSH) is a rare disorder which most commonly occurs in the setting of concurrent lymphoproliferative disease. Morphologically, it consists of aggregates of histiocytes containing eosinophilic crystalline material, which in most cases is composed of aggregated abnormal light chains.
Methods
Using histomorphology, immunohistochemistry and in situ hybridization, the authors characterize a rare case of orbital CSH associated with extranodal marginal zone (MALT) lymphoma and report for the first time the frozen section features of CSH.
Results
The frozen section featured plump histiocytes with ample weakly basophilic to grayish cytoplasm with a microvacuolated appearance and focal stippling. These features stand in contrast with the formalin-fixed, paraffin embedded histomorphological appearance of aggregates of plump histiocytes with densely eosinophilic crystalline cytoplasmic material.
Conclusion
CSH is a challenging diagnosis to make on frozen section. The artifacts that preclude its recognition, as well as differential diagnoses of this entity in the head and neck are discussed.
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MLO and FP conceptualization, data gathering/analysis and writing. BW and HLEC data gathering and critical review of the paper. All authors approved the submitted manuscript and agree to be accountable for all aspects of the work.
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Oon, M.L., Wu, B., Chan, H.L.E. et al. Ocular Crystal-Storing Histiocytosis with Co-existing MALT Lymphoma—A Rare Case with Cytologic and Heretofore Not Reported Findings on Frozen Section. Head and Neck Pathol 17, 1034–1041 (2023). https://doi.org/10.1007/s12105-023-01581-7
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DOI: https://doi.org/10.1007/s12105-023-01581-7