Breast malignant, males, children
Other malignancies
Rosai-Dorfman disease

Author: Monika Roychowdhury, M.D. (see Authors page)

Revised: 27 December 2016, last major update August 2012

Copyright: (c) 2002-2016,, Inc.

PubMed Search: rosai-dorfman disease breast
Cite this page: Rosai-Dorfman disease. website. Accessed October 24th, 2018.
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

Images hosted on PathOut server:

Mammogram, courtesy of 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

Images hosted on PathOut server:

Courtesy of Dr. Mark R. Wick

Erythrophagocytosis, AFIP

With marked sinusoidal edema, AFIP

Sinusoids distended by histiocytes, AFIP

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

Images hosted on PathOut server:

Parenchyma, FNAB, courtesy of Dr. Mark R. Wick

Positive stains
Negative stains