2018 Volume 111 Issue 2 Pages 133-138
Rosai-Dorfman disease (RDD) is a rare proliferative histiocytic disorder characterized by persistent massive lympadenopathy mimicking malignant tumors. Herein, we describe the case of 78-year-old male patient with RDD of the head and neck. The patient was referred to our hospital with a left neck mass. Computed tomographic (CT) examination revealed a subcutaneous tumor on the left side of the neck. 18F-Fluorodeoxyglucose positron emission tomography (PET)/CT revealed elevated uptake in the mass, the ribs and the thoracic vertebrae. Left cervical lymph node biopsy, as part of a whole-body examination performed bearing in mind the possibility of cancer of unknown primary origin, initially revealed no diagnosis other than reactive lymph nodes. Therefore, a thoracic spinal biopsy was additionally performed. When the lymph node biopsy specimens were reviewed again, the findings were found to be consistent with the diagnosis of RDD. RDD is a rare disease characterized by emperipolesis, in which lymphocytes and erythrocytes are taken up, without degeneration, in histiocytic spheres. The patient was then treated with a steroid, which resulted in improvement of both the neck lymph node swelling and the blood findings.
