Ewing Sarcoma is a rare, aggressive malignant tumor originating in bone or soft tissues (ESFT family: Classic bone, Extraosseous, pPNET, Askin tumor).
Genetic Translocations & Causes
Genetic driver: In 85% of cases, chromosomal translocation t(11;22)(q24;q12) creates the EWSR1-FLI1 fusion gene.
Affects children and adolescents (ages 5-25, peak 10-20 yrs). More prevalent in males and European descent.
Symptoms & Anatomical Sites
Sites: Pelvis (most frequent), femur, tibia, ribs (Askin tumor), humerus, spine.
Symptoms: Persistent progressive bone pain, swelling/lump, warmth, fractures, fever, fatigue, weight loss, anemia, night sweats.
Diagnostics, VDC-IE Chemo & Surgery
Diagnostics: X-ray ('onion skin' periosteal reaction), MRI, CT, Bone scan, PET-CT, Biopsy (small round blue cells, CD99+), FISH / RT-PCR genetic testing.
Treatment: Alternating VDC-IE Chemotherapy (Vincristine, Doxorubicin, Cyclophosphamide / Ifosfamide, Etoposide), Wide surgical excision (limb-sparing), Radiotherapy, High-Dose Chemo with Stem Cell Rescue.
Prognosis: 70-80% 5-yr survival for localized disease.