Ewing sarcoma

Case contributed by Dr Abdallah Khateeb

Presentation

6 years old female presenting with right thigh mass and pain.

Patient Data

Age: 6 years old
Gender: Female

CT Scan

Modality: CT

The bone window of this CT scan shows an aggressive type multi-lamellated, elevated and interrupted periosteal reaction.
Endosteal scalloping is also evident and involves most of the femur's diaphysis, but most obvious at the lower third.
A focal area of frank cortical breach is also seen at the medial side of femur, opposite to which the muscles are displaced away by a heterogenous lobulated mass (better seen on the soft tissue window), hinting significant soft tissue involvement and possible acute hemorrhage.

IMPRESSION: aggressive tumor with significant soft tissue component.
DDx: Ewing sarcoma > osteosarcoma >> osteomyelitis > metastasis.

MRI with Gd

Modality: MRI

Enhanced MRI of the region shows the true extent of the tumor, which involves most of the marrow cavity of the femur. The associated large soft tissue mass shows heterogenous mainly preipheral enhancement due to internal necrosis. A large acute bleed into the mass is also seen.

Case Discussion

Ewing sarcoma is an aggressive malignant bone tumor seen in unlucky young subjects.

The multi-lamellated periosteal reaction (aka Onion skinning) is a hallmark of this tumor, though, it is not pathognomonic. 
Diaphyseal origin of the tumor and associated large soft tissue mass and / or swelling are characteristic features of Ewing sarcoma.

 

For this case, tissue biopsy confirmed Ewing sarcoma.

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Case Information

rID: 43287
Case created: 3rd Mar 2016
Last edited: 5th Mar 2016
Inclusion in quiz mode: Included

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