成人眼眶黄色肉芽肿病临床病理分析及文献复习
Adult orbital xanthogranulomatous disease with clinicopathological analysis report and literature review
李桂 1杨艳丽 2李东海 1闫秀英1
作者信息
- 1. 兰州市第一人民医院
- 2. 中国人民解放军940医院病理科,甘肃兰州 730050
- 折叠
摘要
目的 探讨成人眼眶黄色肉芽肿病的临床病理特征、诊断、鉴别诊断及预后.方法 回顾性分析1例56岁男性成人眼眶黄色肉芽肿病(adult orbital xanthogranulomatous disease AOXGD,AOXGD)患者的临床病理资料并复习相关文献进行总结.结果 发现双眼睑肿物7年,伴逐渐增大.巨检:左眼上睑肿物一块,大小1.0cm×0.8cm×0.3cm,切面灰黄质韧,左眼下睑肿物一块,大小3.0cm×2.0cm×0.9cm切面灰黄质中.镜检:病变位于真皮及皮下组织,见多量组织细胞浸润,部分胞浆嗜酸性,核偏位,大部分组织细胞胞浆呈泡沫状,背景伴有不等量淋巴细胞、浆细胞、多核巨细胞(Touton巨细胞),局部骨骼肌内见泡沫样组织细胞浸润.免疫表型:组织细胞CD68、CD163、TREM-1阳性,CD1a、S-100、IgG4、Langerin均阴性,Ki67约5％.BRAF V600E基因检测无突变.结论 AOXGD在临床上较为罕见,病理分型较多,诊断与治疗存在一定困难,容易误诊误治.其中AOX亚型单纯手术切除,预后好,ECD亚型预后最差,伴有BRAF V600E突变患者,可用靶向药物威罗菲尼治疗.TREM-1可能参与泡沫样组织细胞的形成,与AOXGD发生相关,有望成为AOXGD治疗的一个新的靶点或监测AOXGD复发进展的一项新指标.
Abstract
Objective To investigate the clinicopathological features,diagnosis,differential diagnosis and prognosis of adult orbital xanthogranulomatous disease.Methods The clinical and pathology data of one 56 year old male patient with Adult orbital xan-thogranulomatous disease(AOXGD)were retrospectively analyzed,along with a review and summarize of the relevant literatures.Results The patient was male,56 year old.Both eyelid masses were found for 7 years,with gradual enlargement.Giant examina-tion:a upper eyelid mass of the left eye,size 1.0cm×0.8 cm×0.3 cm,section surface gryxanthin tough,left eye eyelid mass,size 3.0 cm×2.0 cm×0.9 cm section gryxanthin.Microscopy:The lesion is located in the subcutaneous tissue with multiple histiocytic infiltration,Partly acidophilic and biased,the majority of histiocytes were foamy,Background with unequal amounts of lympho-cytes,plasma cells,multinucleated giant cells(Duton cells),and foamy id histiocytic infiltration in local skeletal muscle.Immuno-phenotype:histiocytes were positive for CD68 and CD163,TREM-1 negative for CDla,S-100 and Lahrin-,about 5％Ki67.BRAF V600E gene detection without mutation.Conclusion AOXGD is relatively rare in clinical practice,with multiple pathological sub-types,making diagnosis and treatment difficult and prone to misdiagnosis and mistreatment.Among them,AOX subtype with simple surgical resection has a good prognosis,while ECD subtype has the worst prognosis.Patients with BRAF V600E mutation can be treated with targeted drug Verofinib.TREM-1 may participate in the formation of foam like tissue cells and be related to the occur-rence of AOXGD.It is expected to become a new target for AOXGD treatment or a new indicator for monitoring the recurrence and progress of AOXGD.
关键词
成人眼眶黄色肉芽肿病/亚型/免疫组化/鉴别诊断/TREM-1Key words
Adult orbital xanthogranulomatous disease/Subtype/Immunohistochemistry/Differential diagnosis/TREM-1引用本文复制引用
基金项目
兰州市卫生健康行业科研项目(A2023026)
出版年
2024
NETL
NSTL
万方数据