Ewing's Sarcoma: The Daily PANCE Blueprint
A 16-year-old boy presents with left thigh pain that has been worsening over the past two months. He recalls bumping his leg during a basketball game before the pain started. He was seen in the clinic 3 weeks ago and was diagnosed with a muscle strain. On exam, there is a mass palpable over the anterior distal thigh. X-ray of the femur shows a proximal femoral diaphyseal lesion with cortical destruction and a periosteal reaction in an onion-skin pattern. Which of the following is true regarding the most likely diagnosis?
A. It is most commonly found in the elderly
B. It is associated with a t(11;22) translocation
C. It commonly involves the pelvis and ribs
D. It usually presents with systemic symptoms like fever and weight loss
E. Radiation therapy is not part of the treatment
Answer and topic summary
The answer is B. It is associated with a t(11;22) translocation
The most likely diagnosis is Ewing sarcoma, which is characterized by the presence of a t(11;22) translocation. This chromosomal abnormality is a hallmark of Ewing sarcoma and aids in diagnosis. Ewing sarcoma commonly presents with pain and a palpable mass, often in the diaphysis of long bones, with an onion-skin periosteal reaction on imaging.
Incorrect Answer Explanations:
- A. It is most commonly found in the elderly: Ewing sarcoma primarily affects children and young adults, not the elderly.
- C. It commonly involves the pelvis and ribs: While Ewing sarcoma can involve the pelvis and ribs, the most common sites are the diaphysis of long bones, such as the femur.
- D. It usually presents with systemic symptoms like fever and weight loss: Systemic symptoms can occur but are not as common as localized pain and swelling.
- E. Radiation therapy is not part of the treatment: Radiation therapy is often part of the treatment for Ewing sarcoma, along with surgery and chemotherapy.
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