The spectrum of orbital Rosai-Dorfman disease.
OBJECTIVE
To describe the spectrum and treatment of orbital Rosai-Dorfman disease and to review previously documented cases.


METHODS
Retrospective, interventional case series of seven patients and literature review.


RESULTS
Each patient with Rosai-Dorfman disease had unique disease expression requiring aggressive therapy, such as chemotherapy, radiation, and/or surgical excision. One systemically aggressive case presented with intraocular and choroidal invasion, a previously unreported finding. Response to therapy was variable and unpredictable.


CONCLUSIONS
Rosai-Dorfman disease, although historically described as benign and self-limiting, may cause significant morbidity and mortality involving multiple organ systems. Available treatment options may not control the disease. Further research and long-term clinical correlation is necessary.
Mohadjer
Yasaman
Y
Department of Ophthalmology, Saint Louis University, MO 63141, USA,
Holds
John B
JB
Rootman
Jack
J
Wilson
Mathew W
MW
Gigantelli
James W
JW
Custer
Philip L
PL
D000293:Adolescent; D000328:Adult; D005260:Female; D015618:Histiocytosis, Sinus / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D009916:Orbital Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed