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.
Adult orbital xanthogranulomatous diseaseSubtypeImmunohistochemistryDifferential diagnosisTREM-1
NETL
NSTL
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