Rosai dorfman disease of the orbit

Geeta K Vemuganti¹ , Milind N Naik² and Santosh G Honavar²

¹Ophthalmic Pathology Service, Hyderbad Eye Research Centre, L V Prasad Eye Institute, Hyderbad, India

²Division of Ophthalmic Plastic Surgery, Orbit, and Ocular Oncology, LV Prasad Eye Institute, Hyderabad, India

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Journal of Hematology & Oncology 2008, 1:7doi:10.1186/1756-8722-1-7


Published:	28 June 2008

Abstract

Objective

To report the clinico-histopathologic features, management and outcome of Rosai-Dorfman disease of the orbit.

Design

Non-comparative case series.

Results

Rosai-Dorfman disease of the orbit constituted 0.09% of all ocular specimens received at our Institute, presenting with a firm rubbery mass causing proptosis; bilateral in 4 (57%) cases. The median age at presentation was 13 years (range 5–65); median duration of symptoms was 6 (range 3–15) years. Lymphadenopathy was noted in 4 (57%); extranodal involvement in 3 (43%). After biopsy, 3 cases were treated with systemic corticosteroids, 2 cases developed local recurrence that responded to systemic corticosteroid therapy. Polymorphous population of lymphocytes, plasma cells, and characteristic S-100-positive histiocytes showing emperipolesis were pathognomonic histologic features.

Conclusion

Rosai-Dorfman disease of the orbit, although rare, should be considered in young individuals with chronic proptosis with rubbery masses. Excision and corticosteroid therapy provide a favorable outcome.