Abstract

Rosai-Dorfman disease is a rare proliferative histiocytic disorder of unknown etiology. It is a unique disorder of the lymph nodes but extranodal sites such as bone, skin, central nervous system, upper respiratory tract and soft tissue may be affected. Rosai-Dorfman disease is presented with massive, symmetrical cervical lymphadenopathy, fever, leucocytosis, elevated sedimantation rate and hipergamaglobulinemia. Soft tissue presentation can masquerading as a sarcoma. 61 year old women referred with painless left thigh mass to our hospital. After a excisional biopsy histopathologic results showed sheets of histiocytes with emperipolesis admixed in a mixture of inflammatuary cells including plasma cells and lymphocytes. Additionally, we observed pseudovascular spaces and asteroid bodies in large histiocytes. After surgery the patient has been followed up for 1 year postoperatively, without any sign of reccurence. We herein report a case of soft tissue Rosai-Dorfman disease wich presented with unusual features such as pseudovascular spaces and asteroid bodies in histiocytes.

Keywords: Rosai-Dorfman disease; Soft tissue; Pseudovascular spaces; Asteroid bodies