Soft Tissue Rosai-Dorfman Disease with Unusual Histopathologic Features: A Case Report

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.

Rare Presentation of Rosai-Dorfman Disease in Soft Tissue: Diagnostic Findings and Surgical Treatment

“…The differential diagnosis for RDD includes Langerhans cell histiocytosis (LCH), soft tissue fibrohistiocytic lesions, histiocytic sarcomas, and myxoinflammatory fibroblastic sarcomas. LCH may occur in similar sites as RDD, but these lesions exhibit Langerhans cells with grooved nuclei, eosinophils, and Birbeck granules [ 10 ]. Additionally, although LCH also consists of S100+ cells, it is the Langerhans cells—not the histiocytes—that are S100+ in LCH.…”

Enfermedad de Rosai Dorfman y Reactivación de Toxoplasmosis: Presentación de un Caso

“…Otras enfermedades a considerar incluyen las neoplasias histiociticas y el tumor tendosinovial de células gigantes. (Tataroglu et al 2017)…”