Rosai-Dorfman disease:report of seven cases and review of literature
Objective To study the clinicopathological features,diagnosis,and differential diagnosis of Rosai-Dorfman disease(RDD)with review of the relevant literature.Methods A retrospective study was conducted on 7 cases of Rosai-Dorfman disease diagnosed in Panzhihua Central Hospital from 2014 to 2023.All cases were reviewed by three senior pathologists.Results Seven patients were 7-54 years old,including 3 males and 4 females,with a male to female ratio of 3∶4.Among the 7 cases,sites of occurrence included nasal cavity,hip,lateral upper arm of right upper limb,intracranial(left parietal lobe),spinal canal,right breast for one case each,and one case involved both left thigh and lower abdomen.Clinical manifestations varied according to the location of occurrence.Under the microscope,the morphology was roughly the same.A large number of histiocytes,lymphocytes and plasma cells were noted,showing alternating dark areas and bright areas.The emperipolesis(intracytoplasmic lymphocytes)was observed at higher magnifeication.Immunohistochemical staining showed histiocytes were positive for S-100,CD68 and cyclin-D1,but negative for Langerin and AE1/AE3.Conclusion The incidence rate of Rosai-Dorfman disease is low,the age of onset is various,the female patients are more common,the clinical and imaging presentations are not typical,and the diagosis mainly depends on microscopic features and immunohistochemistry.
Rosai-Dorfman diseaseClinicopathological featuresDiagnosis and differential diagnosis