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Treatment of extraskeletal mesenchymal chondrosarcoma
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
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Context: Treatment of Chondro-osseous Tumors / Extraskeletal Mesenchymal Chondrosarcoma
Treatment options for extraskeletal mesenchymal chondrosarcoma include the following:
Surgery.
Chemotherapy followed by surgery and additional chemotherapy. Radiation therapy may also be given.
A review of 15 patients younger than 26 years included 11 patients with soft tissue lesions from the German Cooperative Soft Tissue Sarcoma Study Group and 4 patients with primary bone lesions from the German-Austrian-Swiss Cooperative Osteosarcoma Study Group protocols. The review suggested that complete surgical removal, or incomplete resection followed by radiation therapy, was necessary for local control.[9][Level of evidence C1]
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A single-institution, retrospective review identified 12 pediatric patients with mesenchymal chondrosarcoma.[10] Eleven patients presented with localized disease, and one patient presented with pulmonary nodules. Six patients received preoperative chemotherapy. All patients received postoperative chemotherapy (most commonly ifosfamide/doxorubicin) with or without radiation therapy (median dose, 59.4 Gy).
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The NCOA2 rearrangement was documented in these patients' tumors.
The study confirmed that surgical resection is necessary for cure.
At a median follow-up of 4.8 years, the 5-year DFS rate was 68.2% (95% confidence interval [CI], 39.8%–96.6%), and the OS rate was 88.9% (95% CI, 66.9%–100%).
A Japanese study of patients with extraskeletal myxoid chondrosarcoma and mesenchymal chondrosarcoma randomly assigned patients to treatment with either trabectedin or best supportive care.[11] The median age of patients was 38 years (range, 21–77 years).
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The OS was higher for the patients assigned to receive trabectedin than for patients assigned to receive best supportive care.
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Preserved source evidence · Not medical advice