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← Childhood Soft Tissue Sarcoma

HEALTH PROFESSIONAL · SOURCE READING

Surgery with or without radiation therapy

Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: April 21, 2025 · Captured 2026-09-09.

Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary.

Context: Treatment of Tumors of Uncertain Differentiation / Clear Cell Sarcoma NOS / Treatment of clear cell sarcoma of soft tissue

Surgery with or without radiation therapy is the treatment of choice and offers the best chance for cure.

Evidence (surgery with or without radiation therapy):

In a series of 28 pediatric patients reported by the Italian and German Soft Tissue Cooperative Studies, the median age at diagnosis was 14 years and the lower extremity was the most common primary site (50%).[91]; [92][Level of evidence C2]

Source links and citations

In a series of 28 pediatric patients reported by the Italian and German Soft Tissue Cooperative Studies, the median age at diagnosis was 14 years and the lower extremity was the most common primary site (50%).[91]; [92][Level of evidence C2]

In this series, 12 of 13 patients with completely resected tumors were cured.

In a series of 28 pediatric patients reported by the Italian and German Soft Tissue Cooperative Studies, the median age at diagnosis was 14 years and the lower extremity was the most common primary site (50%).[91]; [92][Level of evidence C2]

For patients with more advanced disease, the outcome is poor and chemotherapy is rarely effective.

Publication references

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Preserved source evidence · Not medical advice