Craniocervical Rosai-Dorfman Disease presentation: Case report and review of literature

Rosai Dorfman Disease (RDD) is a benign histiocytic lymphoproliferative disease that has variable presentations. The concurrent presentation of RDD in the spinal cord and brain parenchyma is an extremely rare entity. Here, we report another case of a 24-year-old gentleman who presented with a tuberculum sellae and bilateral cavernous extra-axial tumors extending to the subtemporal lobe and was found to have craniocervical lesions. Axillary lymph node biopsy was done showing markedly dilated sinuses filled with large histiocytes and emperipolesis of numerous lymphocytes and plasma cells confirming the diagnosis of RDD. Because the definitive diagnosis of RDD is always pathological, the clinical presentation plays a major role in widening the margin of differential diagnosis. Finally, surgical intervention is the first option to treat RDD with relatively satisfactory follow-up outcomes, and other adjuvant therapies optimize the prognosis.