A case of orbital Rosai-Dorfman disease responding to radiotherapy.
Rosai-Dorfman disease (RDD) is a rare histiocytic disorder most often characterized by painless cervical lymphadenopathy, but it may also present with orbital disease. The clinical course of RDD is variable; it can be either relapsing-remitting or progressive, and the outcome relates to clinical location and treatment response. Orbital RDD can have an insidious onset and similar presentation to other ophthalmic conditions; this can result in a delayed diagnosis. Nearly all cases of orbital RDD cause visual disturbances and require treatment. Because orbital RDD is an uncommon presentation, a variety of interventions have been employed, including surgery, immunotherapy, chemotherapy, and radiotherapy. We present a case of salvage radiotherapy for progressive orbital RDD refractory to surgery and chemotherapy in a pediatric patient.
Cooper
Samuel Lewis
SL
Department of Radiation Oncology, Medical University of South Carolina, Charleston, SC, USA.
Chavis
Pamela S
PS
Fortney
John A
JA
Watkins
John M
JM
Caplan
Michael J
MJ
Jenrette
Joseph M
JM
D000293:Adolescent; D005260:Female; D015618:Histiocytosis, Sinus / Q000175:diagnosis / Q000532:radiotherapy*; D006801:Humans; D008279:Magnetic Resonance Imaging; D009916:Orbital Diseases / Q000175:diagnosis / Q000532:radiotherapy*; D016879:Salvage Therapy; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014786:Vision Disorders