Research
Rosai dorfman disease of the orbit
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* Corresponding author: Geeta K Vemuganti geeta@lvpei.org
1 Ophthalmic Pathology Service, Hyderbad Eye Research Centre, L V Prasad Eye Institute, Hyderbad, India
2 Division of Ophthalmic Plastic Surgery, Orbit, and Ocular Oncology, LV Prasad Eye Institute, Hyderabad, India
Journal of Hematology & Oncology 2008, 1:7 doi:10.1186/1756-8722-1-7
Published: 28 June 2008Abstract
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.