A case of orbital Rosai-Dorfman disease responding to radiotherapy

J Pediatr Hematol Oncol. 2008 Oct;30(10):744-8. doi: 10.1097/MPH.0b013e31817e4ac1.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Female
  • Histiocytosis, Sinus / diagnosis
  • Histiocytosis, Sinus / radiotherapy*
  • Humans
  • Magnetic Resonance Imaging
  • Orbital Diseases / diagnosis
  • Orbital Diseases / radiotherapy*
  • Salvage Therapy
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Vision Disorders