ROSAI-DORFMAN DISEASE WITH MAMMARY INVOLVEMENT: CASE REPORT

Camargo Júnior, H. S. A. (CDE Diagnóstico por Imagem, Campinas, SP, Brasil.), Teixeira, S. R. C. (CDE Diagnóstico por Imagem, Campinas, SP, Brasil.), Arruda, M. S. (CDE Diagnóstico por Imagem, Campinas, SP, Brasil.), Negrão, E. M. S. (CDE Diagnóstico por

CDE Diagnóstico por Imagem, Campinas, SP, Brasil.We present a rare breast manifestation of Rosai-Dorfman disease (DRD), which is an idiopathic benign proliferative histiocytic disease of unknown origin, described in 1969. Clinical history: 57-year-old patient with a rapidly growing palpable nodule. Mammography and ultrasound: voluminous lesion with imprecise borders. US guided biopsy: chronic mastitis with fibrosis and lymphoid hyperplasia. Magnetic resonance imaging: suspicious finding of a contrast enhancing mass. Immunohistochemical and morphological analysis of surgical specimen showed DRD associated with sclerosing disease related to IgG4. Also known as sinus histiocytosis with massive lymphadenopathy, DRD may have extra nodal manifestations, but breast involvement is very rare. The most common clinical presentation includes painless bilateral cervical lymphadenopathy, fever, and weight loss. It tends to resolve spontaneously, but may require surgery or radiation therapy. In addition, there may be generalized nodal and extra nodal spread and even fatality. Awareness of the many rare diseases that can eventually cause breast manifestations is important. The DRD in this case mimicked the carcinoma in its clinical and radiographic presentation, causing a diagnostic dilemma. The result of the percutaneous biopsy also illustrated the difficulty of the differential histological diagnosis with other benign or malignant histiocytic lesions. The correct diagnosis in this patient was fundamental for proper treatment.ROSAI-DORFMAN

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