Orthopedic Oncology cases Ewing sarcoma

A 15-year-old female is diagnosed with Ewing sarcoma of the tibia.

 

Which of the following is true regarding the treatment and prognosis of Ewing sarcoma?

1. Local control of the primary tumor can be achieved by either wide resection or external beam radiation

2. There is no role for chemotherapy in treatment of small, round blue cell tumors

3. Five-year survival for patients with isolated extremity Ewing sarcoma is 10% to 15%

4. Ewing sarcoma is one of the few musculoskeletal malignancies that can metastasize via lymph nodes

Discussion

The correct answer is (A). The standard treatment algorithm for Ewing sarcoma includes neoadjuvant chemotherapy followed by either resection or radiation therapy. Most isolated extremity lesions are treated with surgical resection rather than radiation, because complications of radiation in skeletally immature individuals include joint contractures, fibrosis, growth arrest, fracture, and secondary malignancy. Patients with isolated extremity Ewing sarcoma have a 5-year survival of 65% to 70%, and when it does metastasize, it does via hematogenous spread to the lungs, bone, or bone marrow.

Which of the following is a prognostic indicator for overall survival in Ewing sarcoma?

1. Periosteal reaction

2. Percent of necrosis after chemotherapy

3. Patient age

4. Intensity of radiotracer uptake on bone scan

Discussion

The correct answer is (B). Response to neoadjuvant chemotherapy (percent necrosis) is used as a prognostic indicator for overall survival in Ewing sarcoma. All Ewing sarcomas display intense radiotracer uptake on bone scan, and periosteal reaction is a nonspecific finding that appears in radiographs of many aggressive tumors. Patient age has not been shown to correlate with overall prognosis.

 

Objectives: Did you learn...?

 

Ewing sarcoma treatment?

 

Ewing sarcoma prognosis and staging?