Primary Bilateral Intraosseous Rosai-Dorfman Disease

Area of Science:

• Histiocytic Disorders
• Bone Pathology
• Rare Diseases

Background:

• Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder of unknown cause.
• Bone involvement in RDD is uncommon, typically occurring in less than 10% of cases.
• Primary intraosseous RDD is exceptionally rare.

Observation:

• This report describes the first documented case of primary, bilateral intraosseous Rosai-Dorfman disease.
• The condition presented asymmetrically, affecting the lateral femoral condyles in a 59-year-old male.
• The lesion was incidentally discovered during the workup for a giant cell tumor of the bone in the tibia.

Findings:

• Diagnosis required multiple biopsies and extensive evaluation due to the rarity and unusual presentation.
• Key clinical, radiological, and pathological findings were crucial for establishing the diagnosis.
• Differential diagnostic considerations were significant given the lesion's appearance.

Implications:

• This case expands the understanding of Rosai-Dorfman disease presentation in bone.
• Highlights the importance of considering RDD in the differential diagnosis of intraosseous lesions, even with atypical presentations.
• Emphasizes the diagnostic challenges and the need for thorough evaluation in rare bone diseases.