Ewing's Sarcoma

Ewing's Sarcoma

Male predominance
Peaks during adolescent growth spurt
More common than osteosarcoma when patient is < 10 y/o
Rare in African-Americans and Asians
t11:22 chromosome translocation

Clinical Presentation

Pain, swelling, limitation of movement
50% will have palpable mass
Fever and weight loss (more so than osteosarcoma)
Which part of the bone & which bones?
Diaphysis of long bones
Distal femur>proximal tibia> proximal humerus
Central axis (pelvis, spine & chest wall): “Askin tumor”
Metastasis to lungs, bone, bone marrow, & lymph nodes

Imaging

X ray
Destructive diaphyseal lesion with erosion of the cortex and spread to surrounding soft tissue
“Onion skinning”
CT to define bony extent
MRI superior to define extent of marrow and soft tissue involvement

Treatment

Chemotherapy
Very sensitive to radiotherapy
Surgical excision vs. radiotherapy??

Prognosis

60-75% survival: small, isolated, extremity tumor
< 30% survival for metastatic disease

Osteosarcoma -- Ewing's

Age: 2nd decade -- 2nd decade
Associations: Rb, Li-Fraumeni, radiotherapy -- none known
Site of presentation: metaphyses of long bone -- diaphyses of long bone
X-ray pattern from extension into soft tissue: starburst -- onion-skinning

CHLA Board Review 2005