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Rosai-Dorfman Disease of the Breast

¹ Division of Diagnostic Imaging, University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd., Box 57, Houston, TX 77030.

Received February 9, 2005; accepted after revision March 23, 2005.

Address correspondence to G. J. Whitman (gwhitman{at}di.mdacc.tmc.edu).

A 53-year-old woman presented with an ill-defined mass in the left breast on screening mammography. There was no evidence of a palpable abnormality, skin changes, or nipple discharge. Laboratory tests revealed a mild normocytic anemia and an elevated erythrocyte sedimentation rate. Mammography (Fig. 1A) of the left breast showed a 1.5-cm high-density mass with indistinct margins in the 7:00 position, 8 cm from the nipple. Sonography (Fig. 1B) showed an ill-defined, hypoechoic mass, which correlated with the mammographic finding. A sonography-guided needle core biopsy was performed, followed by a stereotactic vacuum-assisted core biopsy. Histopathology (Fig. 1C) revealed a polymorphous infiltrate of histiocytes, mature-appearing lymphocytes, and plasma cells surrounded by areas of fibrosis. The histiocytes were large with round nuclei, a single eosinophilic nucleolus, and abundant pale cytoplasm. Many of the histiocytes contained lymphocytes in cytoplasmic vacuoles, a finding referred to as emperipolesis. Immunohistochemical stains performed on fixed, paraffin-embedded tissue sections showed that the histiocytes expressed S-100 protein, characteristic of Rosai-Dorfman disease. The clinicopathologic diagnosis was Rosai-Dorfman disease.

View larger version (82K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A —53-year-old woman with Rosai-Dorfman disease of breast. Left breast craniocaudal mammogram shows 1.5-cm high-density mass with indistinct margins (arrow).

View larger version (117K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B —53-year-old woman with Rosai-Dorfman disease of breast. Left breast longitudinal sonogram shows ill-defined, hypoechoic mass (arrow).

View larger version (162K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1C —53-year-old woman with Rosai-Dorfman disease of breast. Photomicrograph shows histiocytes with emperipolesis (arrowhead) in background of lymphocytes, plasma cells, and fibrosis. (H and E, x500)

Rosai-Dorfman disease is an idiopathic benign proliferative disorder of histiocytes. First described by Rosai and Dorfman in 1969, it is also known as sinus histiocytosis with massive lymphadenopathy [1]. Extranodal sites are involved in up to 43% of the cases, with 23% of cases having only extranodal involvement. Common sites of extranodal involvement include the skin and the subcutaneous tissues, the upper airway, and the salivary glands [1]. Breast involvement is rare. Nine cases have been previously reported to involve the breast parenchyma [2, 3]. Six additional cases have involved the skin of the breast [2].

The classic presentation for Rosai-Dorfman disease is painless bilateral cervical lymphadenopathy in a young asymptomatic patient. Most patients have an elevated erythrocyte sedimentation rate. Less commonly, patients may have anemia, leukocytosis, and polyclonal hypergammaglobulinemia [2]. In most patients, the lesions regress spontaneously. However, patients may develop recurrence, persistent but stable disease, or progressive disease. In rare cases, a patient may die because of Rosai-Dorfman disease [4]. Widespread organ involvement and immunologic abnormalities portend a poor prognosis [5].

Patients with breast involvement usually present with a palpable mass, tenderness, or an abnormal mammogram. Mammograms show an ill-defined mass without calcifications. Sonography usually shows a hypoechoic mass.

Rosai-Dorfman disease is defined by its histopathologic features, which are similar regardless of the site of occurrence. The hallmark of Rosai-Dorfman disease is a large, polygonal histiocyte with a round nucleus, vesicular chromatin, fine nuclear membrane, one to several eosinophilic nucleoli, and abundant pale cytoplasm. Many of the histiocytes show emperipolesis, a phenomenon characterized by the presence of lymphocytes within the cytoplasm of other cells [2]. The histiocytes are seen in a background of mature-appearing lymphocytes, plasma cells, and occasionally fibrosis [5]. Emperipolesis is often more difficult to appreciate in extranodal lesions than in lymph nodes. On immunohistochemical stains, the histiocytes of Rosai-Dorfman disease strongly express S-100 protein, unlike typical sinus histiocytes. The histiocytes also express pan-macrophage antigens, markers associated with lysosomal activity, activation antigens, and antigens expressed by monocytes but not by tissue macrophages [3].

The differential diagnosis based on the mammographic and sonographic findings is that of a solid benign or malignant mass. The differential diagnosis based on the histopathologic features includes carcinoma, malakoplakia, diabetic mastopathy, fat necrosis, granulomatous mastitis, and Langerhans' histiocytosis. The diagnosis may be established by fine-needle aspiration, core needle biopsy, or surgical excision. In this case, the patient was diagnosed by core needle biopsy without a need for surgery. Subsequent evaluation showed no other disease sites, and the patient received no additional treatment [2].

References

1. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 1990;7 : 19-73[Medline]
2. Hummel P, Waisman J, Chhieng D, Yan Z, Cohen JM, Cangiarella J. Fine-needle aspiration cytology of Rosai-Dorfman disease of the breast: a case report. Diagn Cytopathol 1999;21 : 287-291[Medline]
3. Ng SB, Tan L, Tan LH. Rosai-Dorfman disease of the breast: a mimic of breast malignancy. Pathology 2000;32 : 10-15[Medline]
4. Warnke RA, Weiss LM, Chan JKC, et al. Tumors of the lymph nodes and spleen. In: Armed Forces Institute of Pathology, Atlas of tumor pathology, 3rd ed., fasc. 14. Washington, DC: AFIP,1995 : 349-360
5. Hammond LA, Keh C, Rowlands DC. Rosai-Dorfman disease in the breast. Histopathology 1996;29 : 582-584[Medline]

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This Article Figures Only Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Pham, C. B. Articles by Stephens, T. W. Search for Related Content PubMed PubMed Citation Articles by Pham, C. B. Articles by Stephens, T. W. Social Bookmarking                      What's this?