CNS tumor
Lymphoma and other hematopoietic lesions
Rosai-Dorfman disease

Topic Completed: 1 January 2006

Revised: 3 January 2019, last major update January 2006

Copyright: (c) 2002-2018,, Inc.

PubMed Search: Rosai-Dorfman[title] CNS

Nat Pernick, M.D.
Page views in 2019: 1,768
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Cite this page: Pernick N. Rosai-Dorfman disease. website. Accessed February 18th, 2020.
Definition / general
  • Also called sinus histiocytosis with massive lymphadenopathy
  • Often (25 - 43%) has extranodal involvement
  • Rare (100 cases/year in US) and isolated CNS findings are extremely rare
  • Benign but may be associated with systemic disease
  • Children and young adults (mean age 41 years, range 22 - 63 years)
  • CNS tumors usually associated with dura
  • References: Mod Pathol 2001;14:172, Clin Neuropathol 2005;24:112 (isolated CNS disease)
Case reports
  • Surgical biopsy or excision
Gross images

Images hosted on other servers:

Extra-axial firm solid mass showing broad dural attachment

Microscopic (histologic) description
  • Proliferation of variably sized, pale staining histiocytes, the larger ones may exhibit emperipolesis (engulf intact lymphocytes)
  • Often extensive lymphoplasmacytic infiltrate and collagen
Microscopic (histologic) images

Images hosted on other servers:

Fig 1: MRI shows extra-axial mass with adjacent edema;
Fig 2: inflammatory infiltrate with histiocytes, lymphocytes and plasma cells;
Fig 3: histiocytic emperipolesis; fig 4: S100+ histiocytes

Various images

Cytology description
  • Rosai-Dorfman histiocytes (voluminous pale pink cytoplasm, single or multiple nuclei, large and hyperchromatic, with lymphagocytosis)
  • Also scattered lymphoid aggregates with loose mixture of lymphocytes, plasma cells and classic histiocytes (Acta Cytol 2003;47:1111)
Positive stains
Negative stains
Differential diagnosis
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