Potential role of 18F-FDG PET/CT in a case of progressive Rosai Dorfman disease

Rosai Dorfman disease is a rare form of nonlangerhans cell histiocytosis, presenting with extensive lymphadenopathies. Treatment in most cases of nodal disease, involves close observation; however, extranodal involvement requires a more definitive treatment strategy. Herein, we report a case of extensive Rosai Dorfman disease in a 43-year-old woman presenting for evaluation of treatment response by 18F-FDG PET/CT after frequent relapses and disease progression. In addition to extensive lymphadenothapies in cervical, supraclavicular, superior mediastinum, axillary, abdominopelvic and inguinofemoral regions, the patient had metabolically active bone and subcutaneous lesions which were not previously recognized. Following this 18F-FDG PET/CT study, the patient management was changed to sirolimus and prednisolone. To choose the best treatment option for Rosai Dorfman patients, knowledge of the full extent of disease is important. Compared with conventional imaging, 18F-FDG PET/CT has the advantage of being a whole-body imaging modality and can recognize disease involvement prior to any anatomical changes.