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Case Reports
. 2017 Apr 11;9(4):e1153.
doi: 10.7759/cureus.1153.

Rosai-Dorfman Disease of the Breast

Eileen E Delaney  1 , Anne Larkin  2 , Sue MacMaster  1 , Ali Sakhdari  3 , Carolynn M DeBenedectis  1
Affiliations
Case Reports

Rosai-Dorfman Disease of the Breast

Eileen E Delaney et al. Cureus. .

Abstract

Rosai-Dorfman disease (also known as sinus histiocytosis with massive lymphadenopathy) is a rare benign proliferative disorder of histiocytes that typically involves the lymph nodes and can also involve extranodal sites. Rosai-Dorfman disease confined to the breast is extremely rare, but important to recognize as it can mimic malignancy. We present the case of a 63-year-old woman who presented with a palpable breast lump that was highly suspicious for malignancy based on mammogram and ultrasound appearance. Biopsy revealed inflammatory tissue with lymphoplasmacytic and histiocytic predominance. The diagnosis of Rosai-Dorfman was made based on characteristic staining of histiocytes with S-100 and the presence of emperipolesis. Early recognition of this benign disease entity spared the patient further investigation and surgical intervention.

Keywords: breast; inflammatory disorder; non-malignant breast disease; rosai-dorfman; sinus histiocytosis with massive lymphadenopathy.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Full field digital diagnostic mammogram demonstrates focal asymmetry in the upper outer quadrant of the right breast corresponding to the palpable marker.
Figure 2
Figure 2. Grayscale and color ultrasound images demonstrate a solid mass with an irregular shape and surrounding edema at site of mammographic and clinically palpable mass, 11 o’clock 9 cm from the nipple.
Figure 3
Figure 3. Breast biopsy demonstrating sheets of medium to large histiocytic cells staining diffusely and strongly with S100 protein (100X, 200X).
Figure 4
Figure 4. Breast parenchyma with sheets of medium to large histiocytic cells demonstrating faint staining with CD68 (100X).
Figure 5
Figure 5. Full field digital diagnostic mammogram and spot compression views demonstrate a focal asymmetry without calcifications.
Figure 6
Figure 6. Grayscale and color ultrasound images demonstrate a solid mass with internal vascularity at 1 o’clock 13 cm from the nipple.
Figure 7
Figure 7. Breast tissue totally replaced by sheets of Rosai-Dorfman histiocytes, small lymphocytes, and scattered plasma cells. Many histiocytes show the emperipolesis of lymphocytes (H&E stain, 400X).
H&E - Hemotoxylin and Eosin
See this image and copyright information in PMC

References

    1. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Rosai J, Dorfman R. https://www.ncbi.nlm.nih.gov/pubmed/5782438. Arch Pathol. 1969;87:63–70. - PubMed
    1. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Foucar E, Rosai J, Dorfman R. https://www.ncbi.nlm.nih.gov/pubmed/2180012. Semin Diagn Pathol. 1990;7:19–73. - PubMed
    1. Rosai-Dorfman disease of the breast. Pham CB, Abruzzo LV, Cook E, et al. AJR Am J Roentgenol. 2005;185:971–972. - PubMed
    1. Rosai-Dorfman disease presenting as a breast mass and enlarged axillary lymph node mimicking malignancy: a case report and review of the literature. Tenny SO, McGinness M, Zhang D, et al. Breast J. 2011;17:516–520. - PubMed
    1. Rosai-Dorfman disease confined to the breast. Morkowski JJ, Nguyen CV, Lin P, et al. Ann Diagn Pathol. 2010;14:81–87. - PubMed

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