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Treatment Option Overview for Childhood Soft Tissue Sarcoma
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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Because of the rarity of pediatric nonrhabdomyosarcomatous soft tissue sarcomas (NRSTS), treatment should be coordinated by a multidisciplinary team that includes oncologists (pediatric or medical), pathologists, surgeons, and radiation oncologists for all children, adolescents, and young adults with these tumors. In addition, to better define the tumors' natural history and response to therapy, entry into national or institutional treatment protocols should be considered for children with rare neoplasms. Information about ongoing clinical trials is available from the NCI website.
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The Children's Oncology Group (COG) performed a prospective nonrandomized trial (ARST0332 [NCT00346164]) for patients with soft tissue sarcomas.[1]
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Surgical resection of the primary tumor was classified as follows:
R0 if the resection was complete with negative microscopic margins.
R1 if the margins were microscopically positive.
R2 if the resection left macroscopic residual tumor.
Patients were assigned to one of the following three risk groups:
Low risk: Nonmetastatic R0 or R1 low-grade, or ≤5 cm R1 high-grade tumor.
Intermediate risk: Nonmetastatic R0 or R1 >5 cm high-grade, or unresected tumor of any size or grade.
High risk: Metastatic tumor.
The treatment groups were as follows:
Surgery alone.
Radiation therapy (55.8 Gy).
Chemoradiation therapy (chemotherapy and 55.8 Gy radiation therapy).
Neoadjuvant chemoradiation therapy (chemotherapy and 45 Gy radiation therapy, then surgery and radiation therapy boost based on margins with continued chemotherapy).
Chemotherapy included six cycles of intravenous (IV) ifosfamide (3 g/m2 per dose) on days 1 through 3 and five cycles of IV doxorubicin (37.5 mg/m2 per dose) on days 1 to 2 every 3 weeks, with the sequence adjusted based on timing of surgery or radiation therapy.
The analysis included 529 evaluable patients: low risk (n = 222), intermediate risk (n = 227), and high risk (n = 80). Patients underwent surgery alone (n = 205), radiation therapy (n = 17), chemoradiation therapy (n = 111), and neoadjuvant chemoradiation therapy (n = 196).
At a median follow-up of 6.5 years (interquartile range [IQR], 4.9–7.9), the 5-year event-free survival (EFS) and overall survival (OS) rates, by risk group, were as follows:
Low-risk group: EFS rate, 88.9% (95% confidence interval [CI], 84.0%–93.8%) and OS rate, 96.2% (95% CI, 93.2%–99.2%).
Intermediate-risk group: EFS rate, 65.0% (95% CI, 58.2%–71.8%) and OS rate, 79.2% (95% CI, 73.4%–85.0%).
High-risk group: EFS rate, 21.2% (95% CI, 11.4%–31.1%) and OS rate, 35.5% (95% CI, 23.6%–47.4%).
The authors concluded that pretreatment clinical features can be used to effectively define treatment failure risk and stratify young patients with NRSTS for risk-adapted therapy. Most low-risk patients can be cured without adjuvant therapy, avoiding known long-term treatment complications. Survival remains suboptimal for intermediate-risk and high-risk patients, and novel therapies are needed for these patients.
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