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


METHODS
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.


CONCLUSIONS
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.
Vemuganti
Geeta K
GK
Ophthalmic Pathology Service, Hyderbad Eye Research Centre, L V Prasad Eye Institute, Hyderbad, India. geeta@lvpei.org
Naik
Milind N
MN
Honavar
Santosh G
SG
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D015618:Histiocytosis, Sinus / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery*; D006801:Humans; D008875:Middle Aged; D009916:Orbital Diseases / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery*; D016896:Treatment Outcome; D055815:Young Adult
D000305:Adrenal Cortex Hormones