Purpose: 

To report a rare case of crystal-storing histiocytosis associated with solitary extramedullary plasmacytoma of the lacrimal sac and to review literature on the 2 entities to summarize important diagnostic, management, and prognostic considerations.

Methods: 

A case report of the ophthalmologic presentation, pathology workup, and oncologic management is presented. Literature search with focus on lesions occurring in ophthalmic sites and management guidelines from expert panels and working groups.

Results: 

Crystal-storing histiocytosis associated with solitary extramedullary plasmacytoma arose within the lacrimal sac of a previously healthy middle-aged woman and presented as a painless nodule with epiphora. The biopsy tissue showed sheets of crystal-filled histiocytes, interspersed with monoclonal plasma cells and rarely demonstrated plasma cell phagocytosis. Imaging and laboratory studies confirmed the localized nature.

Conclusions: 

Crystal-storing histiocytosis is an uncommon entity in which crystals, most commonly arising from altered immunoglobulins, aggregate within histiocytes and form symptomatic mass lesions. It has been reported in ophthalmic regions in patients with a concurrent lymphoproliferative or plasma cell disorder and can rarely predate a malignancy. The current case is notable because crystal-storing histiocytosis occurs with a localized process, solitary extramedullary plasmacytoma, and presents in an unusual site, the lacrimal sac. Tissue biopsy with multimodal pathological evaluation is necessary to make the diagnosis. Ophthalmologists should recognize that crystal-storing histiocytosis is commonly associated with a hematologic malignancy and, when appropriate, refer the patient for oncologic management. Surveillance may be indicated in cases with no established etiology. Solitary extramedullary plasmacytoma should also be monitored, as a proportion of cases progress to multiple myeloma.

中文翻译：

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孤立性泪囊髓外浆细胞瘤伴相关晶体储存组织细胞增多症

目的： 

报告一例与泪囊孤立性髓外浆细胞瘤相关的晶体储存组织细胞增多症的罕见病例，并回顾有关这两种疾病的文献，总结重要的诊断、治疗和预后考虑因素。

方法： 

介绍了眼科表现、病理检查和肿瘤治疗的病例报告。文献检索重点关注眼科部位发生的病变以及专家小组和工作组的管理指南。

结果： 

与孤立性髓外浆细胞瘤相关的晶体储存组织细胞增多症发生在一名先前健康的中年妇女的泪囊内，表现为无痛性结节伴溢泪。活检组织显示充满晶体的组织细胞片，散布着单克隆浆细胞，很少表现出浆细胞吞噬作用。成像和实验室研究证实了局部性质。

结论： 

晶体储存组织细胞增多症是一种罕见的实体，其中晶体（最常见的是由改变的免疫球蛋白产生）在组织细胞内聚集并形成有症状的肿块病变。据报道，在患有并发淋巴组织增生或浆细胞疾病的患者的眼部区域中，这种情况很少发生在恶性肿瘤之前。目前的病例值得注意，因为晶体储存组织细胞增多症是伴随局部过程（孤立性髓外浆细胞瘤）发生的，并且出现在一个不寻常的部位，即泪囊。做出诊断需要组织活检和多模式病理学评估。眼科医生应认识到晶体储存组织细胞增多症通常与血液恶性肿瘤相关，并在适当时将患者转诊至肿瘤治疗。对于没有确定病因的病例，可能需要进行监测。还应监测孤立性髓外浆细胞瘤，因为部分病例进展为多发性骨髓瘤。