Rosai-Dorfman disease

Abstract

Objectives: To document the clinicopathological features of Rosai-Dorfman disease (RDD) at 2 tertiary hospitals in the western region of Saudi Arabia. Methods: We retrieved all cases diagnosed as RDD at King Abdulaziz University Hospital and King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia, diagnosed between January 2001 until June 2021. Results: A total of 13 new RDD cases were reported, including 7 nodal and 6 extranodal type. The extranodal sites included larynx, optic chiasm, dura and brain, lumbar vertebrae, and left arm soft tissue. There were 6 males and 7 females. Ages averaged 34 years and ranged from 4-56 years. A total of 2 cases were associated with Hodgkin's lymphoma, and 2 cases have been initially misdiagnosed as other entities. All patients were treated with surgical excision, and steroid was added in 3 cases. Over 2-60 months of follow-up, recurrence occurred in 2 cases. Conclusion: Awareness of this entity is important for pathologists to avoid misdiagnosis. While the optimal treatment remains controversial, surgical resection is generally curative. The prognosis is good with rare recurrence. Multicenteer prospective studies are probably the best to evaluate treatment options and improve outcomes.