An Intractable Intra-Axial central nervous system Rosai-Dorfman Disease: A Case Report

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that often presents with fever, painless cervical lymphadenopathy and leukemia. Most cases of RDD have a favorable outcome. RDD involving only the central nervous system (CNS) is very rare, and intra-axial CNS RDD is little. We report a case of intra-axial intractable CNS RDD with an atypical presentation. The case reported here is that of a woman in her 60s. Magnetic resonance imaging (MRI) showed a solid tumor 30 mm in diameter in the right frontal lobe. Because the tumor was in the primary motor area, a biopsy was performed for diagnosis. The tumor was ultimately diagnosed as RDD. Although the steroids and cladribine temporarily reduced the size of the tumor, it was ultimately unable to control the growth of the tumor. The patient finally died about two and a half years after the onset. In previous reports, two cases of death directly caused by intra-axial CNS RDD had been reported in the past, because in both cases surgical treatment was not possible. In our case, the tumor was in primary motor area and hard to resect. Total surgical resection is likely to be required to cure intra-axial CNS RDD.