Department of Pathology, National University Health System, 5 Lower Kent Ridge Road, Singapore, 119074, Singapore.
Department of Haematology-Oncology, National University Cancer Institute, 5 Lower Kent Ridge Road, Singapore, 119074, Singapore.
Head Neck Pathol. 2023 Dec;17(4):1034-1041. doi: 10.1007/s12105-023-01581-7. Epub 2023 Oct 4.
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.
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.
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.
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.
结晶贮积性组织细胞增生症（CSH）是一种罕见的疾病，最常发生于同时存在淋巴增生性疾病的情况下。从形态学上看，它由含有嗜酸性结晶物质的组织细胞聚集组成，这些结晶物质在大多数情况下由聚集的异常轻链组成。
作者利用组织形态学、免疫组织化学和原位杂交，对一例罕见的眼眶 CSH 伴结外黏膜相关边缘区（MALT）淋巴瘤进行了特征描述，并首次报道了 CSH 的冷冻切片特征。
冷冻切片显示组织细胞饱满，胞浆弱嗜碱性至灰蓝色，具有微空泡外观和点状颗粒。这些特征与福尔马林固定、石蜡包埋的组织形态学表现形成对比，后者显示为密集嗜酸性结晶细胞质物质的饱满组织细胞聚集。
CSH 是冷冻切片诊断的一个具有挑战性的问题。讨论了可能导致其无法识别的假象，以及头颈部该病变的鉴别诊断。
