27 August 2025
: Case report 
Extranodal Rosai-Dorfman Disease in a Pediatric Patient: A Case Report
Challenging differential diagnosis, Rare disease
Lili Miles ABCDEF 1*, Caroline Baughn
ABCDEF 2, Gleidson Messias Silva BD 3, Dorothea L. Douglas ABDE 4, Lei Shao BDEF 1
DOI: 10.12659/AJCR.948533
Am J Case Rep 2025; 26:e948533
Table 1 Clinical, radiological, and pathological features of differential diagnosis of osseous Rosai-Dorfman disease.
| Differential diagnosis | Radiological features | Cultural results | Histological features | Immunohistochemistry profile |
|---|---|---|---|---|
| Ewing sarcoma | Aggressive permeative lesion with onion-skin periosteal reaction and soft tissue mass | Negative | Small blue cells with high grade features | CD1A (−) and S-100 (−) |
| Langerhans cell histiocytosis | Lytic lesion, well-defined, beveled edges in skull or vertebra plana in spine | Negative | Langerhans cells in the background of mixed inflammatory cells and osteoclasts | CD1A (+) and S-100 (+) |
| Osteomyelitis | Lytic lesion with periosteal reaction; possible sequestrum and soft-tissue swelling | Positive | Mixed inflammatory cells with necrotic bone | CD1A (−) and S-100 (−) |
| Extranodal Rosai-Dorfman disease | Lytic lesion with variable sclerosis; can mimic malignancy or infection | Negative | Large histiocytes with emperipolesis | CD1A (−) and S-100 (+) |