Rosai-Dorfman Disease: Importance of ¹⁸F FDG PET/CT to Determine Extension and Extranodal Involvement

Rosai-Dorfman disease or sinus histiocytosis with lymphadenopathy is a rare benign histiocytic proliferative disorder of unknown etiology first described in 1969. It typically affects older females and most common presentation is with massive lymphadenopathy and nonspecific systemic symptoms; therefore, it is often confused with lymphoproliferative disorders [1, 2]. We present the case of a 69-year-old woman with nasal obstruction as only complaint. Laboratory tests showed normal leukocyte count with elevated ANC (absolute neutrophil count), normal RBC count with normal MCV (mean corpuscular volume) and MCH (mean corpuscular hemoglobin), elevated ESR (erythrocyte sedimentation rate), and normal IgG, IgA, and IgM values. Evaluation revealed a nasopharyngeal mass, which was biopsied and reported emperipolesis with positive CD68 and S-100; typical and differential findings of this disease [1, 2]. ¹⁸F FDG PET/CT was performed to determine the extent and involvement of the disease. Considering the presence of few symptoms and no significant laboratory abnormality, treating physicians decided to start a regimen of corticosteroids (prednisolone) for a period of 4 months, after which a follow-up with ¹⁸F FDG PET/CT will be performed.