Orbital Rosai-Dorfman Disease: A Literature Review

Abstract

Introduction: Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with a predilection for the head and neck. Isolated orbital involvement without systemic clinical features is very uncommon.

Background: Classic histopathologic features include phagocytized but intact lymphocytes within large histiocytes; a conditioned termed emperipolesis. Immunohistochemical stains are strongly positive for S-100 and CD68 but negative for CD1a in most cases. Orbital cases are often amenable to complete surgical resection.

Conclusion: RDD should be considered in the differential diagnosis of a young patient in good health who presents with a marked but benign enlargement of an orbital mass. A review of the literature regarding this disease entity and its orbital manifestations is discussed.

• Emperipolesis
• orbital mass
• Rosai-Dorfman-Destombes disease
• sinus histiocytosis with massive lymphadenopathy

Acknowledgements

The authors would like to thank Cindy Kirker, the Madigan Army Medical Center librarian, for her invaluable assistance with background research and support of this project.