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.

Figure 1

Bilateral upper eyelid and preseptal orbital mass in a 16-year-old female with complete mechanical ptosis (1a). Published with permission from Elsevier. This figure was published in the Clinical Ophthalmic Oncology, Vemuganti GK, Honavar SH, Eyelid Stroma Tumors, Page 105, Copyright Elsevier 2007. Three-month post-operative appearance following complete excision of the mass (1b).

Figure 2

The gross specimen of the excised mass with lobulated and smooth surface (2a). The cut section of the specimen shows a solid appearance with a few yellowish areas (2b).

Figure 3

Histopathologic section showing a lymphoid follicle (arrow) surrounded by a cuff of lymphocytes and plasma cells with a few pale areas consisting of sheets of histiocytes (asterisk) (× 50, hematoxylin, eosin).

Figure 4

Histopathologic section showing a polymorphous population of cells consisting of mature lymphocytes and plasma cells interspersed with histiocytes. Histiocytes are large with a vesicular nuclei and abundant cytoplasm with engulfed lymphocytes and plasma cells, a phenomenon called lymphophagocytosis or emperipolesis, a hallmark of Rosai-Dorfman disease (arrow) (× 500, hematoxylin, eosin). Published with permission from Elsevier. This figure was published in the Clinical Ophthalmic Oncology, Vemuganti GK, Honavar SH, Eyelid Stroma Tumors, Page 105, Copyright Elsevier 2007.

Figure 5

Histopathologic section showing large histiocytes with emperipolesis, immunoreactive for S-100 antigen. (× 200, DAB).

Figure 6

Imprint cytology shows large histiocytes (arrow) with vesicular nucleus and phagocytosed lymphocytes and plasma cells (asterisk) within the cytoplasm (× 500, Giemsa).