The spectrum of orbital Rosai-Dorfman disease

PURPOSE: To describe the spectrum and treatment of orbital Rosai-Dorfman disease and to review previously documented cases. METHODS: Retrospective, interventional case series of seven patients and literature review. RESULTS: Each patient with Rosai-Dorfman disease had unique disease expression requiring aggressive therapy, such as chemotherapy, radiation, and/or surgical excision. One systemically aggressive case presented with intraocular and choroidal invasion, a previously unreported finding. Response to therapy was variable and unpredictable. CONCLUSIONS: Rosai-Dorfman disease, although historically described as benign and self-limiting, may cause significant morbidity and mortality involving multiple organ systems. Available treatment options may not control the disease. Further research and long-term clinical correlation is necessary.