y most commonly occurs in children and adolescents

aged 4-15 years. y Males are affected more frequently than females, with a ratio of approximately 1.5:1. y Slow growing tumour. y Common site is the lower end of femur.

pathology
y Bone affected 1. long bones( 2/3 of cases)mainly femur and tibia. 2. 1/3 of cases in flat bones- pelvis, and calcaneum. y Site : diaphysis of the long bone( common site)

y Gross pathology:

Gray white. 2. Soft and thin , almost like pus. 3. The bone may be expanded and the periosteum elevated with subperisosteal new bone formation often in layers. 4. The tumour rupture through cortex and extends into the soft tissues
1.

y Histopathology :

Comprises of sheets of uniform , small cells, resembling lymphocytes. 2. The tumour cells surround a central clear area, forming a pseudo- rossette. 3. The chromatin is finely dispersed usually with no nucleoli and a variable number of mitotic figures. 4. Reticulin is scant in most cases of Ewing sarcoma.
1.

The lesion is composed of small blue round cells.

Clinical features
y pain, which is initially intermittent but becomes y y y y y y y

intense Neurologic signs such as nerve root signs and cord compression. remittent fever mild anemia leukocytosis an elevated erythrocyte sedimentation rate (ESR) Increased serum lactic dehydrogenase (LDH) weight loss

Radiological features
y Lytic lesion in the medullary zone of the midshaft of a

long bone, with cortical destruction and new bone formation in layers- Onion appearance. y In atypical case, the tumour may be located in the metaphysis( confused with osteomyelitis) y It may have soft tissue component with little cortical destruction.( resemble a soft tissue sarcoma)

Ewing sarcoma of femur. Frontal radiograph and lateral radiographs of the femur demonstrate mottled, osteolytic lesion (blue circle) with poorly marginated edges in the diaphysis of the bone. There is sunburst periosteal reaction (red circle) and lamellated periosteal reaction (white arrows).

Differential diagnosis
y Small cell osteosarcoma y Mesenchymal chondrosarcoma y Lymphoma y Metastatic neuroblastoma y peripheral neuroectodermal tumors (PNET)

treatment
y Control of local tumour by radiotherapy( 6000 rads) y Control of metastasis by chemotherapy. y Chemotherapy consists of vincristine ,

cyclopohsphomide, Adriamycin in cycles, repeated every 3-4 weeks for about 12-18 cycles.

prognosis
y Very poor. y Bone to bone secondaries is very common. y With potent chemotherapeutic drugs, 5

years

survival has improved to 30 -40 %