Question
A 12 year old boy presented with localized pain and tenderness in the thigh bone.He has complain of fever.On examination of blood ,ESR was found elevated.Frontal and lateral view of Xray is given in the image.
What can be the most probable diagnosis?(Red circle,blue circle and white arrows are made in the X ray to look for important findings for diagnosis)
A. Osteosarcoma
B. Giant Cell Tumor
C. Ewing’s Sarcoma
D. Osteoid Osteoma
Show Answer
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Correct Answer » C Explanation |
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Ans:C. Ewing’s Sarcoma
The Image shows:
- Frontal radiograph and lateral radiographs of the femur demonstrating 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)
Ewing’s Sarcoma
- Ewing sarcoma is one of the small, round cell lesions of bone
- Second most common malignant bone tumor in children (after osteosarcoma)
- More common in males than females
- Occurs between the ages of 5-30 years
Location
- Arise in medullary cavity, usually of long bones in the lower extremities
- Most commonly occurs in long bones and pelvis
- Most occur in femur, pelvis, tibia, and humerus
- But they can occur in virtually any bone
- Commonly involves metadiaphysis of long bones
Clinical Findings:
- Most common symptoms are localized pain and swelling
- Additional symptoms/signs may include
- Fever
- Weight loss
- Anemia
- Leukocytosis
- Elevated erythrocyte sedimentation rate
Imaging Findings:
- Most lesions are visible on conventional radiographs
- However, their degree of spread is better evaluated with MRI
Common manifestations on conventional radiography include
- Poorly marginated, lytic, destructive lesion
- Permeative (small holes) or moth-eaten (mottled) appearance
- Rarely, they can be sclerotic
- Soft tissue mass or infiltration is common
- Soft tissue mass may occur without destruction of cortex
- Soft tissue mass may produce saucerization (scalloped depression in cortex)
- Periosteal reaction is common
- Lamellated – onion-skinning due to successive layers of periosteal development
- Sunburst or spiculated – hair-on-end appearance when new bone is laid down perpendicular to cortex along Sharpey’s fibers
- Codman’s triangle – formed between elevated periosteum with central destruction of cortex
- Osteosclerosis may be present secondary to reactive bone formation
Prognosis
- 60-75% five-year survival.
Treatment:
- Systemic chemotherapy is the mainstay of treatment with surgery and/or radiotherapy playing a role depending of the location and size of the tumour.