Temporal bone manifestation of primary extranodal Rosai-Dorfman disease: a case report

J Med Case Rep. 2023 Jun 21;17(1):280. doi: 10.1186/s13256-023-03790-8.

Abstract

Background: Rosai-Dorfman disease is a rare benign histiocytic disorder characterized in most cases by painless cervical adenopathy. Less than 10% of extranodal cases involve bony lesions. Primary bone Rosai-Dorfman disease in the absence of nodal disease is extremely rare.

Case presentation: A 48 year-old Caucasian male presented with progressive right-sided otalgia, tinnitus, vertigo, and hearing loss. A right temporal bone lytic lesion was detected on diagnostic imaging. Resection of the lesion and histopathological examination revealed Rosai-Dorfman disease.

Conclusions: Rosai-Dorfman disease primary bone lesions are an atypical presentation of a rare disease. This is the second reported case of Rosai-Dorfman disease arising within the temporal bone. This case study reveals that Rosai-Dorfman disease should be considered for patients presenting with inflammatory/lytic lesions of the temporal bone, in cases where infection and malignancy have been excluded.

Keywords: Case report; Histiocytosis; Rosai–Dorfman disease; Temporal bone.

Publication types

  • Case Reports

MeSH terms

  • Histiocytosis, Sinus* / diagnostic imaging
  • Histiocytosis, Sinus* / surgery
  • Humans
  • Lymphadenopathy*
  • Male
  • Middle Aged