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Intranodal Rosai-Dorfman Disease and its Extranodal Recurrence: A Rare Case Review
Sucheta Gandhe1, Yogesh Pawar1, Chaitanya Borde2
1Department of Pathology, HCG Manavata Cancer Centre, Nashik, 422011 Maharashtra India.
Summary
Rosai-Dorfman disease (RDD), a rare histiocyte disorder, presented as an isolated kidney mass in a 49-year-old male. Diagnosis was confirmed through systematic investigation and clinicopathological evaluation post-nephrectomy.
Area of Science:
- Histiocytic Disorders
- Oncology
- Nephrology
Background:
- Rosai-Dorfman disease (RDD) is a rare, benign histiocyte proliferative disorder.
- Typically presents with lymphadenopathy, but extranodal involvement can occur.
- Isolated renal manifestation of RDD is exceptionally uncommon.
Purpose of the Study:
- To report a unique case of extranodal Rosai-Dorfman disease presenting as an isolated renal mass.
- To highlight the diagnostic challenges and importance of comprehensive evaluation in such rare presentations.
- To contribute to the literature on renal manifestations of RDD.
Main Methods:
- Case presentation of a 49-year-old male with a renal mass.
- Description of the diagnostic process, including systematic investigation and clinicopathological evaluation.
- Details of the surgical management (nephrectomy).
Main Results:
- An isolated renal mass was identified in the patient.
- Post-nephrectomy, clinicopathological evaluation confirmed Rosai-Dorfman disease.
- The patient's extranodal presentation was successfully diagnosed.
Conclusions:
- Extranodal Rosai-Dorfman disease can manifest as an isolated renal mass.
- Thorough clinicopathological assessment is crucial for diagnosing rare conditions like renal RDD.
- This case underscores the diverse clinical spectrum of Rosai-Dorfman disease.

