Rosai-Dorfman disease

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare benign idiopathic proliferative disease that involves phagocytic histiocytes.

The disease predominantly occurs in young adults with a mean age at presentation of 21 years. There may be a slight male predominance 7.

A vast majority of patients (~80%) present with painless massive cervical lymphadenopathy, with associated fever, malaise, weight loss and night-sweats 14

In individuals with intracranial involvement, headaches and seizures have been described and in many, no systemic manifestations are present 8,14. Additional site-specific signs and symptoms may also be present (e.g. pituitary dysfunction). 

The exact cause is unknown but disordered immune regulation and viral infections (e.g. EBV, HHV) are thought to be involved 9.

There is a very wide distribution. The condition can affect a multitude of organ systems which include:

  • nodal involvement
  • extranodal involvement (~30% 9)
    • lungs (~2.5%): nodules and perilymphatic interstitial thickening 9
    • skin
    • nasal cavity
    • orbit: ~7%
    • bone
    • intracranial and spinal disease: rare

Histologically, it is characterised by an attenuated infiltration of lymphoplasmacytic cells and histiocytes of varying size. The large, pale histiocytic cells contain what looks like engulfed lymphocytes ("emperipolesis") within their cellular borders.

  • S100: positive
  • CD11c: positive
  • CD68: positive
  • L1 antigen: positive 
  • CD1a: negative

Due to the wide disease spectrum, radiographic features can be variable. Nodal involvement is appreciated as lymphadenopathy on plain film and cross-sectional imaging.

  • central nervous system 8
    • hyperattenuating meningeal-based mass showing contrast enhancement
    • parenchymal oedema surrounding the lesion may be present 
  • central nervous system​ 8,14
    • ​​meningeal-based masses
      • T1: isointense to grey matter
      • T2: hypointense to grey matter
      • T1 C+ (Gd): homogeneous enhancement

Often shows increased uptake with Gallium scanning and increased metabolism with FDG-PET.

Rosai-Dorfman disease usually follows a benign and self-limiting course with treatment largely targeted at controlling local manifestations (surgical intervention) 6.

This condition was initially described by Juan Rosai and R F Dorfman in 1969 3.

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Article information

rID: 12299
Sections: Pathology, Syndromes
Synonyms or Alternate Spellings:
  • Sinus histiocytosis with massive lymphadenopathy
  • Rosai-Dorfmann disease (RDD)
  • Rosai-Dorfmann disease
  • Rosai-Dorfman disease (RDD)

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