Rosai-Dorfman disease presenting in the oropharynx

Head Neck. 2011 Nov;33(11):1660-3. doi: 10.1002/hed.21446. Epub 2010 Jun 11.

Abstract

Background: We report a case of Rosai-Dorfman disease (RDD) presenting as an oropharyngeal mass, and we provide a detailed discussion of this rare clinical entity.

Methods: An initial biopsy, described as being consistent with lymphoma, was reviewed at a second outside institution and was thought to be reactive lymphoid hyperplasia. The patient then presented to our institution for a second opinion. Direct laryngoscopy revealed a firm 1- to 2-cm lesion involving the left soft palate and superior tonsillar pillar.

Results: The diagnosis of RDD was confirmed with immunohistochemical staining on both cytology and histology. The patient elected conservative management and has remained asymptomatic for over 15 months.

Conclusion: The diagnosis of RDD may be challenging in cases presenting initially with extranodal disease. Close follow-up and repeat biopsies may be necessary. Identification of Rosai-Dorfman cells with emperipolesis and confirmation with appropriate immunohistochemical staining on both cytology and histology is diagnostic of RDD. Conservative treatment is appropriate in select cases.

Publication types

  • Case Reports

MeSH terms

  • Biopsy, Needle
  • Female
  • Follow-Up Studies
  • Histiocytosis, Sinus / diagnosis
  • Histiocytosis, Sinus / drug therapy
  • Histiocytosis, Sinus / pathology*
  • Humans
  • Immunohistochemistry
  • Laryngoscopy / methods
  • Lymph Nodes / pathology*
  • Magnetic Resonance Imaging / methods
  • Middle Aged
  • Monitoring, Physiologic / methods
  • Oropharynx / pathology*
  • Prednisone / therapeutic use*
  • Rare Diseases
  • Risk Assessment
  • Severity of Illness Index
  • Tomography, X-Ray Computed / methods
  • Treatment Outcome

Substances

  • Prednisone