Rosai-Dorfman disease (RDD), originally described as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Patients usually present with massive cervical lymphadenopathy. However other nodal sites (mediastinal and inguinal) are also frequently involved. The retro-peritoneum is an infrequent site of involvement. This uncommon entity should also be included in the list of differential diagnoses of diffuse and massive lymphadenopathy, which includes includes infectious/granulomatous conditions, lymphomatous and metastatic disease.
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Primary intraosseous Rosai-Dorfman disease: Clinicopathological features and an assessment of a possible relationship with IgG4-related disease.
Ann Diagn Pathol
January 2025
Department of Pathology, Peking University People's Hospital, 100044, China.
Rosai-Dorfman disease (RDD) is a rare proliferative disorder of histiocytes, and primary solitary RDD of the bone is extremely rare. Some RDDs exhibit increased immunoglobulin (Ig)G4 positive (IgG4+) plasma cell infiltration and the histopathological features of IgG4-related disease (IgG4-RD). However, the association between RDD and IgG4-RD remains unclear.
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Ann Thorac Surg Short Rep
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Division of Cardiac Surgery, University of British Columbia, Vancouver, British Columbia, Canada.
Rosai-Dorfman disease (RDD) is a nonmalignant disease of histiocyte proliferation. RDD usually presents with painless cervical lymphadenopathy, although extranodal involvement can occur. Cardiac involvement was reported in <0.
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Eur J Breast Health
January 2025
Department of Radiology, Miller School of Medicine, University of Miami, Miami, FL, USA.
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).
View Article and Find Full Text PDFAchievement of Clinical Remission in Life-Threatening Rosai-Dorfman Disease With Cladribine.
Cureus
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Hematology and Medical Oncology, Kettering Health, Kettering, USA.
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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