Rosai-Dorfman Disease of the Breast with Variable IgG4+ Plasma Cells: A Diagnostic Mimicker of Other Malignant and Reactive Entities

Jenny C. Hoffmann, Chieh Yu Lin, Siddhartha Bhattacharyya, Olga K. Weinberg, Karen M. Chisholm, Michael Bayerl, Michael Cascio, Girish Venkataraman, Kimberly Allison, Megan Troxell, Chung Che Chang, Adam Bagg, Tracy I. George, Dennis O'Malley, Robert S. Ohgami

Research output: Contribution to journalArticlepeer-review

18 Scopus citations


Rosai-Dorfman disease (RDD) is an uncommon disorder, characterized by an atypical expansion of histiocytes which classically shows emperipolesis and immunoreactivity with S-100 protein. RDD affects the lymph nodes as well as extranodal sites; however, RDD of the breast is exceptionally rare. Herein, we describe the histopathologic features of 22 cases of RDD occurring in the breast, with an emphasis on the differential diagnosis. All cases were notable for an exuberant lymphocytic infiltrate with and without germinal center formation, and the majority (19/22) showed numerous plasma cells: 5 to 132/high-power field (HPF). IgG and IgG4 immunohistochemical stains were available for 13 cases; in no instance were criteria for IgG4-related sclerosing disease met, though in a single case the IgG4/IgG ratio was increased to 25%. Sclerosis was present in the majority of cases (18/22), and was frequently prominent. RDD cells showing emperipolesis were present in all cases (22/22), and ranged from rare (<1/50 HPF) to numerous (>50/50 HPF). Two of the cases in our series were initially misdiagnosed as inflammatory myofibroblastic tumor and plasma cell mastitis with granulomatous inflammation. As emperipolesis can be indistinct, the presence of stromal fibrosis and a prominent lymphoplasmacytic inflammatory infiltrate should prompt a careful search for the characteristic histiocytes, which can be aided by the use of S-100 immunohistochemistry.

Original languageEnglish (US)
Pages (from-to)1653-1660
Number of pages8
JournalAmerican Journal of Surgical Pathology
Issue number12
StatePublished - Dec 1 2019
Externally publishedYes


  • IgG4-related disease
  • Rosai-Dorfman disease
  • myofibroblastic tumor

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine


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