探讨眼眶朗格汉斯细胞组织细胞增生症(LCH)的临床特征。

回顾性病例分析。收集2013年1月至2015年6月于中国人民解放军总医院第三医学中心眼眶研究所确诊21例(21只眼)眼眶LCH患者的临床病历资料。其中，男性14例(14只眼)，女性7例(7只眼)；年龄1~51岁，平均年龄(7.4±10.0)岁。检查并记录所有患者的年龄、性别、临床表现、病灶位置、影像学、病理组织学、治疗及预后的结果。临床资料使用频数和百分比进行描述。

所有患者主要临床表现为额部或颧面部肿块，部分患者伴有炎症表现，眼球突出。病变位于右眼眶者8例(8只眼)，占38.10%(8/21)；左眼眶者13例(13只眼)，占61.90%(13/21)；眼眶外上壁交界处者6例(6只眼)，占28.57%(6/21)；外侧壁者5例(5只眼)，占23.81%(5/21)；上壁者9例(9只眼)，占42.56%(9/21)；外下壁交界处者1例(1只眼)，占4.76%(1/21)。电子计算机断层扫描检查发现所有患者有溶骨性骨质破坏，磁共振成像有17例(17只眼)表现为T1WI中信号，T2WI中高混杂信号，增强后多呈不均匀强化，占80.95%(17/21)；部分肿瘤内部可见不规则增强区。病理组织学检查发现了典型的朗格汉斯细胞，且伴有出血。免疫组织化学检查发现S-100蛋白染色在20例(20只眼)中均可见肿瘤细胞浆和细胞膜呈阳性表达；Ki-67抗原阳性者仅1例(1只眼)，占4.76%(1/21)。21例(21只眼)均行手术治疗，切除的肿瘤体积大小不一，形态不规则，无纤维性包膜。随访3个月至1年，仅1例复发，占4.76%(1/21)。

眼眶LCH以眶周软组织肿块或眼球突出为主要表现，影像学检查是重要的诊断方法，确诊需结合免疫组织化学检查。手术切除是首选的治疗方法，眼眶LCH单系统受累患者预后较好，但应密切观察，长期随访。

To explore the clinical features of orbital Langerhans cell histiocytosis (LCH).

Retrospective cases series. Clinical data of 21 cases (21 eyes) patients with orbital LCH in the Orbital Research Institute of the 3rd Medical Center of the People′s Liberation Army Hospital from January 2013 to June 2015 were selected. Among of them, there were 14 males (14 eyes) and 7 females (7 eyes), aged from 1 to 51 years-old with the average age of (7.4±10.0) years-old. Age, sex, clinical presentation, lesion location, imaging findings, and histopathological results, treatment method and prognosis were recorded. The clinical data were described by using frequency and percentage.

All patients mainly showed lumps at frontal part or cheek, inflammatory infiltration and protopsis. Among of them, 8 cases (8 eyes) accouting for 38.10% (8/21) were found lesions at the right orbit, 13 cases (13 eyes) accouting for 61.90% (13/21) at the left orbit, 6 cases (6 eyes) accouting for 28.57% (6/21) at the upper exterior boundary of orbit, 5 cases (5 eyes) accouting for 23.81% (5/21) at lateral wall, 9 cases (9 eyes) accouting for 42.56% (9/21) at upper wall, and 1 case (1 eye) accouting for 4.76%(1/21) at lower exterior boundary. The osteolytic bone destruction was founded by computed tomography, T1WI middle-intensity signals and T2WI middle-high-intensity mixed signals was founded by magnetic resonance imaging in 17 cases (17 eyes) accouting for 80.95% (17/21) . These signals were unevenly strengthened after the enhancement with irregular strengthened zone of tumor interior. Typical LCH with hemorrhage was found by histopathological examination. 20 cases (20 eyes) were showed tumor cytoplasm and positive cytomembrane after S-100 dyeing. Only one case (one eye) was showed Ki-67 antigen positive rate accouting for 4.76% (1/21). 21 cases (21 eyes) were treated by tumorectomy. The excision tumors had uneven size and shapes without fibroid diolame. After 3 months to 1 year follow-up visits, only one case accouting for 4.76% (1/21) showed recurrence.

Orbital LCH was manifested by orbital lumps or exophthalmos. Imaging examination was an important diagnosis method, and accurate diagnosis was based on immunohistochemical examination. Excision was the first choice in the treatment. Patients with orbital single-system LCH have good prognosis, but follow-up analysis is needed.