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← Childhood Soft Tissue Sarcoma

HEALTH PROFESSIONAL · SOURCE READING

Treatment of desmoplastic small round cell tumor

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 / Desmoplastic Small Round Cell Tumor

There is no standard approach to the treatment of desmoplastic small round cell tumor.

Treatment options for desmoplastic small round cell tumor include the following:

Multimodality therapy (chemotherapy, surgery, and radiation therapy).

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Surgery with hyperthermic intraperitoneal chemotherapy (HIPEC).

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Other treatment options.

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Multimodality therapy →Surgery with HIPEC →Other treatment options →

Preserved source evidence · Not medical advice