Pathophysiology
1) aggressive type of bone neoplasm affecting mostly adolescents 2) considered a type of primitive neuroectodermal tumor of childhood, even though the original neoplastic transformation is probably mesenchymal in origin (possibly in the bone marrow) 3) also occurs in soft tissue 4) 90% of cases associated with the 11/22 chromosome translocation
Signs and Symptoms
1) pain at affected site 2) adjacent soft tissue mass 3) fever 4) most commonly affects long bones (especially humerus, tibia, and femur) 5) metastases are via blood system and go to lungs (most commonly), other bones, and bone marrow 6) patients sometimes give history of mass and pain arising after area has received a trauma
Characteristic Test Findings
Laboratory – 1) leukocytosis Radiology – 2) classic finding is onion peel on plain radiograph owing to reactive periosteal bone 3) soft tissue mass on MRI
Histology
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