Clinicopathological Features, Treatment Response, and Outcome of Rosai-Dorfman Disease in Two Children.

Rosai-Dorfman disease is a rare non-Langerhans cell histiocytic disorder. It is common in male children and young adults of African descent. It is classified into sporadic and familial types. The most common clinical presentation is massive bilateral cervical lymphadenopathy associated with constitutional symptoms. Histiocytic emperipolesis is a diagnostic hallmark for Rosai-Dorfman disease. The diagnostic criteria for Rosai-Dorfman disease are large histiocytic cells that stain positive for CD68 and S100, but negative for CD1a. We present two cases of sporadic Rosai-Dorfman disease in two males of African descent. The first patient had classic nodal Rosai-Dorfman disease; however, he was initially misdiagnosed as lymphoma. He responded poorly to cytotoxic drugs, but achieved a durable complete remission with three cycles of oral prednisolone. The second case had both nodal and extranodal Rosai-Dorfman disease. He initially responded well to steroids, but the response was short-lived. However, he achieved complete remission with a combination of oral methotrexate and vincristine. Rosai-Dorfman disease usually has a simple and uncomplicated clinical course. There is no universal standard guideline for managing Rosai-Dorfman disease. However, patients with symptomatic disease, those experiencing emergency symptoms, and cases of relapse will require medical or surgical intervention to improve their outcomes.