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Rosai dorfman disease of the orbit.

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.

SUBMITTER: Vemuganti GK

PROVIDER: S-EPMC2474646 | biostudies-literature | 2008 Jun

REPOSITORIES: biostudies-literature

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Publications

Rosai dorfman disease of the orbit.

Vemuganti Geeta K GK Naik Milind N MN Honavar Santosh G SG

Journal of hematology & oncology 20080628

<h4>Objective</h4>To report the clinico-histopathologic features, management and outcome of Rosai-Dorfman disease of the orbit.<h4>Design</h4>Non-comparative case series.<h4>Results</h4>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 ...[more]

PMID: 18588698

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