Medical Central Resource

Ewing's sarcoma

Medical Advanced Technology Management Office

Presentation

11 year old male who suffered minor trauma to right leg and was seen in an emergency room where he was noted to have an abnormal plain film of the right tibia and fibula.

Imaging Technique

Plain X-ray MRI

Imaging Findings

The plain films of the right tibia and fibula demonstrate destruction of the proximal fibula with poorly defined margins, violation of the cortical bone and little, if any periosteal reaction.

Diagnosis

Ewing's sarcoma

Discussion

The differential diagnosis of a lytic/destructive lesion in the long bones of a child primarily includes Ewing's sarcoma, Eosinophilic granuloma, osteomyelitis and osteosarcoma. Eosinophilic granuma and osteomyelitis tend to have a less aggressive appearance, so the margins may be better defined and any periosteal reaction will have sharper borders, whereas Ewing's sarcoma and osteosarcomas tend to be more aggressive, so the margins may be more poorly defined. Osteosarcomas usually are bone forming tumors whereas Ewing's is not.
This was a case of Ewing's sarcoma.

EWING's SARCOMA:
4-10% of all bone tumors (behind osteosarcoma and chondrosarcoma)

Histologically composed of "small, round blue cells" which histologically appear similar to lymphoma, osteosarcoma, myeloma, neuroblastoma, carcimoma and EG. There is usually periostitis and a large soft tissue component. Glycogen granules are present and alkaline phosphatase is absent.

AGE: 5 months to 54 years with a peak at 15 years.
Symptoms include local pain, soft tissue mass, fever, leukocytosis and anemia.
The long bones and flat bone are affected with the tumor almost always being metaphyseal or diaphyseal in the long bones. Radiographically, one can see a long lytic lesion in the shaft of the bone with dense lesions being less common. Later, there is a mottled permeative pattern of destruction. In 55% of cases there is penetration into the soft tissues with preservation of tissue planes.
An "onion-peel" or "sunburst/hair-on-end" periosteal reaction is common. Metastases to the lung or bone are also not uncommon.

Submitted by: Medical Advanced Technology Management Office(MATMO)
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References