Bone and Soft Tissue Sarcomas

Outcomes EWS

• Local Rx only  >80% distant failure
• Combination chemotherapy + local control
• 55-75% EFS – localized tumors
• 20-30% EFS – metastases present at diagnosis
• Patients with spine or paravertebral disease have a slightly worse prognosis overall, as well as a higher rate of local failure and tumor recurrence

Complications / Late effects EWS

• Radiation
• Second malignant neoplasms
• Relatively high rate, especially bone tumors
• Bone growth arrest
• Chemotherapy
• Anthracycline-induced cardiomyopathy
• Veno-occlusive disease of the liver
• Infertility
• Second malignant neoplasms

Bone tumors: “Compare & Contrast”

Epidemiology

• Osteosarcoma
• #1 bone tumor
• 2nd decade of life
• Rare under 10 yr
• M:F 1.5:1
• 15% more common in African-Americans than in Caucasians

• Ewing Sarcoma
• #2 bone tumor
• 2nd decade of life
• Not rare under 10 yr
• M:F ~1.4:1
• Rare in African-Americans, other ethnic groups

Presentation

• Osteosarcoma
• Pain
• Often mass
• Duration of symptoms 3 months

• Ewing Sarcoma
• Pain
• Often mass/swelling
• Duration of symptoms 9 months
• Systemic symptoms
• Fever, weight loss

Associations / Risk Factors

• Osteosarcoma
• Lots
• Radiation
• Hereditary retinoblastoma
• Li-Fraumeni
• Rothmund-Thomson
• Cytogenetics
• None characteristic

• Ewing Sarcoma
• None established
• Cytogenetics
• t(11;22)

Location of disease

• Osteosarcoma
• Majority occur in the distal femur, proximal tibia and proximal humerus
• Mostly metaphyseal
• Most mets to lungs

• Ewing Sarcoma
• Fairly even split between the extremities and the axial skeleton
• Axial occur in pelvis, chest wall, spine, head and neck
• Classically diaphyseal
• Mets to lungs, bone, bone marrow