Rosai-Dorfman Disease Confined to the Breast

Abstract

The Rosai-Dorfman (also known as sinus histiocytosis with massive lymphadenopathy), was described in 1969 by Rosai and Dorfman, and is considered a rare disease of unknown aetiology characterized by benign histiocytic lymphadenopathy in the neck and extranodal sites. The involvement of the breast is rare and often simulates invasive breast carcinoma in its clinical presentation and radiological findings. There are currently about 20 cases reported in the literature, and recognition of this uncommon condition, when it occurs in an atypical location such as breast, is extremely difficult. The research aims to report a case of Rosai-Dorfman breast in a woman aged 37, with ill-defined mass in the left breast. Highlighting the immunohistochemical study of biopsy material, and it diffused expression for S-100 protein in histiocytic cells of the parenchyma.

Rosai–Dorfman disease of the breast: A potential marker of systemic disease

“…Mammogram may show an ill‐defined mass without calcifications . According to the literature, ultrasound usually shows a hypoechoic mass with internal vascularity, similar to our findings . Establishing breast involvement is important because it necessitates establishing the full extent of disease elsewhere in the body.…”

“…Breast involvement is rare, with <40 cases reported in the English literature to date. Patients with RDD and breast involvement typically present with painless palpable breast masses, although several cases have been discovered in asymptomatic women on screening mammogram . Herein, we highlight the importance of recognizing the mammographic features of RDD, which often mimic malignancy, correlation with biopsy results, and recommending further imaging to delineate additional sites of disease.…”

“…The classic presentation for Rosai–Dorfman disease is painless, massive, bilateral cervical lymphadenopathy in a young asymptomatic patient . RDD has been reported in various age groups; however, there is a propensity for young adult males (1.4:1), and commonly the disease occurs in Caucasians and Africans . Additional symptoms including fever, anemia, leukocytosis with neutrophilia, and polyclonal hypergammaglobulinemia, although less common, have also been reported …”

“…Although most of the RDD involvement in the breast is solitary, rare multifocal, and/or bilateral breast involvement has been described . Most often, however, patients with involvement of the breast present with a palpable mass and ill‐defined sensation or abnormal mammogram . Mammogram may show an ill‐defined mass without calcifications .…”

“…Widespread organ involvement and immunologic abnormalities correlate with a poor prognosis and in rare cases may result in death . Currently, there is no worldwide consensus on treatment or follow‐up of RDD …”