Imaging Review of Rosai-Dorfman Disease: Updated Neoplastic Classification and Organ-Based Findings

Rosai-Dorfman disease is now a neoplastic process per 2022 WHO classification, driven by MAPK mutations. Imaging shows painless lymphadenopathy or extranodal masses; nonspecific but guides biopsy and treatment response. Rare, but radiologists should know updated classification a…

• Review of imaging across organ systems, reflecting reclassification from inflammatory to neoplastic based on MAPK pathway mutations.
• Hallmark: massive painless cervical lymphadenopathy; extranodal disease mimics malignancy, requiring biopsy for diagnosis.
• Imaging is nonspecific but essential for disease extent and treatment monitoring; management follows 2016 Histiocyte Society consensus guidelines.

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