Breast malignant
Other malignancies
Rosai-Dorfman disease

Topic Completed: 1 August 2012

Revised: 19 September 2019

Copyright: 2002-2019,, Inc.

PubMed Search: rosai-dorfman disease breast

Monika Roychowdhury, M.D.
Page views in 2018: 7,275
Page views in 2019 to date: 7,595
Cite this page: Roychowdhury M. Rosai-Dorfman disease. website. Accessed October 22nd, 2019.
Definition / general
  • Typically massive lymph node involvement that may also involve extranodal sites
  • Associated with systemic symptoms (fever, leukocytosis, anemia)
  • Not part of WHO breast classification
  • Also called sinus histiocytosis with massive lymphadenopathy
Clinical features
  • Very rare in breast, < 25 cases reported
  • May have disease confined to breast or disseminated disease
  • Most common sites are skin, subcutis, upper respiratory tract, bone, eye, adnexae and CNS
  • Usually resolves spontaneously, although systemic cases may cause death
Case reports
Clinical images

Contributed by Dr. Mark R. Wick


Gross description
  • Circumscribed, often multinodular masses in breast stroma
  • Dermis and subcutis uninvolved
Microscopic (histologic) description
  • Large histiocytes with abundant pale eosinophilic cytoplasm, mildly atypical round vesicular nuclei (Am J Surg Pathol 1997;21:664)
  • Lymphocytophagocytosis (emperipolesis) in background of mature lymphocytes and plasma cells
Microscopic (histologic) images

Contributed by Dr. Mark R. Wick

AFIP images


With marked sinusoidal edema

Sinusoids distended by histiocytes

Case of the Week #302

Images hosted on other servers:

Various images

H&E, histiocytes with emperipolesis


Cytology description
  • Large pale cells with enlarged irregular nuclei, lymphocytes and plasma cells, also fragments of fibrous tissue and calcific debris
  • Only rare lymphophagocytosis (emperipolesis, Diagn Cytopathol 1999;21:287)
Cytology images

Contributed by Dr. Mark R. Wick

Parenchyma, FNAB

Positive stains
Negative stains
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