A 13-year-old boy presents with a lytic lesion at the diaphysis of the femur with a permeative pattern and 'onion peel' periosteal reaction on X-ray. Biopsy shows small round blue cells. Preoperative neoadjuvant chemotherapy is planned. Which regimen is standard for this tumor?

• A Methotrexate, adriamycin, cisplatin (MAP)
• B Cisplatin and gemcitabine
• C R-CHOP (rituximab with CHOP)
• D VAC/IE (vincristine, actinomycin D, cyclophosphamide alternating with ifosfamide, etoposide) ✓

Explanation

The onion-peel periosteal reaction and small round blue cells confirm Ewing's sarcoma. The standard neoadjuvant chemotherapy for Ewing's sarcoma is the VAC/IE regimen (vincristine, actinomycin D, cyclophosphamide alternating with ifosfamide and etoposide). The MAP regimen (methotrexate, doxorubicin, cisplatin) is used for osteosarcoma. R-CHOP is for lymphoma. Tumor necrosis >90% after neoadjuvant therapy (Huvos grading) is a favorable prognostic indicator.

Reference: Maheshwari Essential Orthopaedics, 6th ed.

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Written and medically reviewed by the StethoPrep medical team.