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Published on: May 2, 2014
Rosai-Dorfman Disease: Imaging and Updates
Amar S Shah1, Mohd Javed Saifullah Shaikh2, Nishant Aswani3
1Washington University School of Medicine, St Louis, Mo.
Summary
Rosai-Dorfman disease (RDD), a rare histiocytosis, is now classified as a neoplastic process due to MAPK pathway mutations. Radiologic findings are key for diagnosis, staging, and monitoring treatment response in this challenging condition.
Area of Science:
- Hematology
- Oncology
- Radiology
Background:
- Rosai-Dorfman disease (RDD) is a rare, multisystem non-Langerhans cell histiocytosis.
- Recent discoveries reclassified RDD as a neoplastic process, linked to MAPK pathway mutations, and included in the WHO hematolymphoid tumor classification.
- RDD presents with lymphadenopathy and extranodal infiltrates, often mimicking other conditions.
Purpose of the Study:
- To review the diverse radiologic findings of Rosai-Dorfman disease.
- To discuss differential diagnoses based on an organ system approach.
- To outline current consensus management guidelines for RDD.
Main Methods:
- Review of radiologic manifestations of RDD across various organ systems.
- Correlation of imaging findings with clinical and histologic features.
- Discussion of differential diagnoses and management strategies.
Main Results:
- Radiologic findings, though nonspecific, are crucial for initial detection and guiding biopsies.
- Imaging aids in assessing disease extent, prognosis, and treatment response.
- Coexisting conditions can complicate clinical and radiologic interpretation.
Conclusions:
- Radiology plays a vital role in the diagnosis and management of Rosai-Dorfman disease.
- Interpreting imaging findings in conjunction with histology is essential.
- Standardized management guidelines are evolving for this rare neoplastic disorder.
