成人型眼眶黄色肉芽肿病5例临床病理特征分析

目的:探讨成人型眼眶黄色肉芽肿病(AOXGD)的临床病理特征。方法:收集2015-01/2021-01西安市人民医院(西安市第四医院)术后病理确诊AOXGD,回顾性分析临床病理资料,并复习相关文献。结果:共收集AOXGD 5例,包括3种亚型:成人起病的黄色肉芽肿(AOX)3例,坏死性黄色肉芽肿(NBX)1例,Erdheim-Chester脂质肉芽肿病(ECD)1例。5例患者均为中老年患者,以眼眶局部病变就诊,显微镜下可见眼眶皮肤及皮下组织泡沫样非朗格汉斯组织细胞弥漫或巢状浸润,免疫组化CD68、CD163、FXⅢa、溶菌酶阳性,S100、CD1a、Langerin、HMB-45、MDM2阴性。1例ECD患者以眼眶病变为首发症状,合并长骨、心脏、肾脏、腹膜后病变及右心房假性肿瘤。AOXGD诊断依靠临床表现和病理特征,治疗根据亚型和临床表现选择相应策略,主要为糖皮质激素、免疫抑制剂和手术治疗等。结论:AOXGD在临床上较为罕见,不同亚型各有特点,可表现为眼眶局部发病,也可表现为眼眶病变合并全身系统性疾病。应加强对这一组疾病的认识和鉴别,促进正确诊断和规范治疗。

Clinicopathological characteristics of 5 cases of adult orbital xanthogranulomatous disease

AIM:To examine the clinicopathological characteristics of adult orbital xanthogranulomatous disease(AOXGD).METHODS:From January 2015 to January 2021,the researchers collected postoperative pathological diagnoses cases of AOXGD from Xi’an People’s Hospital(Xi’an Fourth Hospital),retrospectively analyzed clinical pathological data,and reviewed related literature.RESULTS:A total of five AOXGD cases were collected,including three cases of adult-onset xanthogranuloma(AOX),one case of necrobiotic xanthogranuloma(NBX),and one case of Erdheim-Chester disease(ECD).The five patients were composed of middle-aged and elderly patients with local orbital lesions were treated.Microscopic examination revealed that the orbital skin and subcutaneous tissue had foam-like non-Langerhans histiocytes that exhibited diffused or nested infiltration.Immunohistochemistry revealed that the cells were positive for CD68,CD163,FXⅢa,lysozyme,negative for S100,CD1a,Langerin,HMB-45,MDM2.One ECD patient had orbital lesions as the first symptom and exhibited a combination of retroperitoneal lesions,right atrium pseudotumor,and long bones,heart,kidney lesions.The diagnosis of AOXGD depends on clinical manifestations and pathological features.The treatment was based on subtypes and clinical manifestations to select corresponding strategies,which mainly include the administration of glucocorticoids,immunosuppressants,and surgical treatment.CONCLUSION:AOXGD is relatively rare in clinical practice.AOXGD can manifest as a local orbital disease or an orbital disease combined with a systemic disease.In the clinical and pathological work of ophthalmology,the understanding and identification of this group of diseases should be strengthened,and correct diagnosis and standard treatment should be promoted.