Ocular Crystal-Storing Histiocytosis with Co-existing MALT Lymphoma-A Rare Case with Cytologic and Heretofore Not Reported Findings on Frozen Section

BackgroundCrystal-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.MethodsUsing 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.ResultsThe 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.ConclusionCSH 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.

Automated summary

This study characterizes a rare case of orbital crystal-storing histiocytosis associated with extranodal marginal zone lymphoma and reports the frozen section features of crystal-storing histiocytosis for the first time.