title: Extranodal Rosai-Dorfman Disease of the Orbit: Clinical Features of 8 Cases.
abstract: OBJECTIVE
  To report on the presentation, radiography, histology, and treatment of 8 cases of extranodal Rosai-Dorfman disease involving the orbit.
  
  
  METHODS
  Multicenter retrospective case series.
  
  
  RESULTS
  Five males and 3 females had a median age of 10 years (range 2-78 years). Presenting signs and symptoms included proptosis, periorbital pain, palpable mass, blepharoptosis, decreased vision, diplopia, impaired extraocular motility, and afferent pupillary defect. Four patients had bilateral orbital disease, while 4 had unilateral disease. Six cases were extraconal, 1 was intraconal, and 1 was both intra- and extra-conal. Four cases had only extranodal disease without lymphadenopathy (3 of which had localized orbital disease). Diagnosis was confirmed by exam, orbital, and/or systemic radiography, and biopsy in all cases. Treatment strategies included excision or debulking, systemic corticosteroids, chemotherapy, radiotherapy, observation or a combination thereof. At last follow up, 4 patients were disease free, while 4 had residual improved disease.
  
  
  CONCLUSIONS
  Rosai-Dorfman disease of the orbit is a rare clinical entity. Purely extranodal disease is rare, with isolated orbital disease being exceedingly rare. This study is unique in that 4 of 8 patients had strictly isolated extranodal disease of the orbit. A large majority of the cases had disease in the extraconal space, contrasting with previous reports. In addition, lacrimal gland disease, particularly bilateral involvement, was prominent in the current study. Although there is no consensus on treatment, surgical excision should be attempted if plausible in symptomatic patients especially if the orbit represents a localized site of disease.
authors.affiliation: *Department of Ophthalmology, SUNY Downstate Medical Center, Brooklyn, New York; †Department of Ophthalmology, Rutgers Biomedical and Health Sciences, Newark, New Jersey; ‡Texas Oculoplastic Consultants, Austin, Texas; §OculoFacial Plastic & Reconstructive Surgery Division, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York; ‖Department of Pathology, New York Eye & Ear Infirmary, New York, New York; ¶Department of Ophthalmology, Emory University Hospital, Atlanta, Georgia; and #Texas Tech University Health Sciences Center, El Paso, Texas, U.S.A.
authors.forename: Jeffrey J
authors.initials: JJ
authors.lastname: Tan
authors.forename: Sherry
authors.initials: S
authors.lastname: Narang
authors.forename: Baljeet
authors.initials: B
authors.lastname: Purewal
authors.forename: Paul D
authors.initials: PD
authors.lastname: Langer
authors.forename: Sean
authors.initials: S
authors.lastname: Blaydon
authors.forename: Robert M
authors.initials: RM
authors.lastname: Schwarcz
authors.forename: Steven A
authors.initials: SA
authors.lastname: McCormick
authors.forename: Joon Y
authors.initials: JY
authors.lastname: Kim
authors.forename: Joseph D
authors.initials: JD
authors.lastname: Walrath
authors.forename: Benjamin O
authors.initials: BO
authors.lastname: Burt
authors.forename: Roman
authors.initials: R
authors.lastname: Shinder
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D005260:Female; D015618:Histiocytosis, Sinus / Q000175:diagnosis*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009915:Orbit / Q000000981:diagnostic imaging*; D009916:Orbital Diseases / Q000175:diagnosis*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D055815:Young Adult