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Rosai–Dorfman Disease: A Rare Clinicopathological Presentation and Review of the Literature
Authors:Aileen Azari-Yaam  Mohammad Reza Abdolsalehi  Mohammad Vasei  Moeinadin Safavi  Mehrzad Mehdizadeh
Affiliation:1.Molecular Pathology and Cytogenetics Division, Department of Pathology, Children’s Medical Center, Tehran University of Medical Sciences, No 63, Gharib Ave, Keshavarz Blv, Tehran, 1419733151 Iran ;2.Department of Infectious Diseases, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran ;3.Department of Radiology, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
Abstract:
Rosai–Dorfman disease (RDD) is a rare and self-limiting disease process that presents most commonly in young patients as massive, painless, cervical lymphadenopathy. Extranodal involvement may also occur. Histopathologic evaluation is the main diagnostic modality. We report an unusual presentation of RDD with cervical lymphadenopathy and an incidentally discovered sinonasal mass, clinically worrisome for malignancy. We emphasize that a high index of clinical suspicion is critical for accurate diagnosis of RDD. Clinicians and pathologists should consider RDD in a differential diagnosis of cervical lymphadenopathy, especially in young patients.
Keywords:Rosai–  Dorfman disease, Sinus histiocytosis with massive lymphadenopathy, Cervical lymphadenopathy, Emperipolesis, Sinonasal mass, Immunohistochemistry, Lymphophagocytosis
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