Ewing sarcoma
Ewing sarcoma
Updated: 08/12/2022
© Jun Wang,
MD, PhD
General features
- AKA primitive neuroectodermal tumor (PNET)
- #2 bone sarcoma in children, after osteosarcoma
- Usually whites, ages 5-20 years
- Possible multiple lesions at presentation
- 5 year survival near 75%
Clinical presentations
- Pain, fever, weight loss
Most common sites
- Medullary cavity, usually shaft
- Femur, tibia, humerus, etc
Key pathogenesis
- EWS-Fli1 activates Cyclin D and Cyclin E
- Activation of Cyclin D and Cyclin E promotes cell cycle progression through suppression of tumor suppressor RB
Key radiologic findings
- Mottled, osteolytic lesion with sunburst periosteal reaction
- Lamellated periosteal reaction
- Codman’s triangle: Shadow between cortex and raised ends of periosteum (due to reactive bone formation), non-specific (can be seen in osteosarcoma)
Key morphological features
- Sheets of small blue cells with scant cytoplasm and round nuclei
Markers
- Positive for CD99
Differential diagnosis
- Lymphoma: Positive for CD45, CD20 if B cell, CD3, etc if T-cell, but negative for CD99
- Small cell carcinoma: Positive for cytokeratin and neuroendocrine markers such as chromogranin, synaptophysin and CD56
Genetic abnormalities
- t(11,22)(q24;q12)/EWS-FLI1
Treatment
- Surgery
- Chemotherapy
- Radiation
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