Rosai-Dorfman disease (RDD) is a rare idiopathic benign proliferative disorder of histiocytes. RDD typically presents with cervical lymph node involvement; however, extranodal sites such as the breast can also be involved and should prompt evaluation for additional sites of disease.
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Rosai-Dorfman disease (RDD) is a self-limited, idiopathic, non-neoplastic disorder characterized by the proliferation of phagocytic histiocytes, which can mimic malignant lymphoproliferative disease. Cases of RDD most commonly present as bilateral painless cervical lymphadenopathy, with lesser involvement of the axilla, inguinal, and mediastinal lymph nodes. We present the case of a 62-year-old woman with a history of endometrial serous carcinoma who underwent evaluation at a dedicated breast imaging department after positron emission tomography/computed tomography (PET/CT) revealed breast masses and axillary nodes with increased uptake of fluorodeoxyglucose (FDG).
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Rosai-Dorfman disease (RDD) is a rare proliferative histiocytic disorder characterized by sinus histiocytosis with massive lymphadenopathy, rarely presenting with severe and life-threatening extra-nodal features. The rarity of RDD, clinically variant phenotype, limited data, and lack of a current standardized management approach make treatment decisions difficult. Herein, we present a case of life-threatening, disseminated RDD with rare clinical features of recurrent pericardial effusion, bilateral pleural effusions, and abdominal tissue fibrosis successfully treated with six cycles of cladribine, achieving clinical remission.
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