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- arises from vascular endothelium of bone
- affects children and teenagers
- men > women
- most frequently affects
- diaphysis of long bones
- pelvis
- ribs
- widespread metastases frequent
- bone marrow is often involved
Clinical features
Histology
- small, darkly staining undifferentiated cells
- neuroendocrine origin
Investigations
- raised ESR
- confusion with osteomyelitis
- X-ray
- large, soft tissue mass
- concentric layers of new bone formation
- "onion skin" appearance
- characteristic chromosomal abnormality
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