Home   Chapter Home   Jobs   Conferences   Fellowships   Books


Breast malignant, males, children

Other malignancies

Rosai-Dorfman disease

Reviewer: Monika Roychowdhury, M.D. (see Reviewers page)
Revised: 27 February 2014, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.


● 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

● 35 year old man (Acta Cytol 2010;54:349)
● 43 year old woman with enlarging breast mass (Univ Pittsburgh Case #338)
● 45 year old woman presented with a right breast mass (Case of the Week #302)
● 50 year old woman with disease confined to breast mimicking cancer (Pathol Res Pract 2007;203:741)
● 65 year old woman with multiple breast nodules (Am J Surg Pathol 1999;23:359)
● 67 year old woman (Gac Med Mex 2010;146:212)
● 75 year old woman with 2 breast tumors (Ned Tijdschr Geneeskd 2011;155:A3176)
● Breast mass and axillary lymphadenopathy (Breast J 2011;17:516)
● Bilateral breast involvement by disseminated Rosai-Dorfman disease (Breast J 2011;17:309)
● Three patients with Rosai-Dorfman disease confined to breast (Ann Diagn Pathol 2010;14:81)
● With recurrence after excision (Am J Med Sci 2010;339:282)

Gross description

● Circumscribed, often multinodular masses in breast stroma
● Dermis and subcutis uninvolved

Micro 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

Micro images

Lymphoplasmacytic infiltrate             Histiocytes with emperipolesis (arrowhead) with
with histiocytes (fig 1)                  lymphocytes, plasma cells and fibrosis

AFIP images:
Erythrophagocytosis                 With marked sinusoidal edema

Emperipolesis        CD68+ histiocytes (fig 2)  Sinusoids distended by histiocytes

Case of the Week #302

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)

Positive stains

● S100, CD68

Negative stains

● CD1a

Differential diagnosis

Idiopathic granulomatous mastitis
Tuberculosis or other infectious granulomas
Langerhans’ cell histiocytosis
Erdheim-Chester disease
Benign fibrous histiocytoma

End of Breast malignant, males, children > Other malignancies > Rosai-Dorfman disease

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).