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← Ewing Sarcoma and Undifferentiated Small Round Cell Sarcomas of Bone and Soft Tissue

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Response to initial therapy factors

Source: Ewing Sarcoma and Undifferentiated Small Round Cell Sarcomas of Bone and Soft Tissue Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: November 27, 2024 · Captured 2026-09-09.

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Multiple studies have shown that patients with minimal or no residual viable tumor after presurgical chemotherapy have a significantly better EFS than do patients with larger amounts of viable tumor.[21,73-76]; [77][Level of evidence C2] In particular, patients with localized disease who have no viable tumor seen at the time of local-control surgery appear to have markedly favorable outcomes.[21]; [77][Level of evidence C2] Female sex and younger age predict a good histological response to preoperative therapy.[78] For patients who receive preinduction- and postinduction-chemotherapy PET scans, decreased PET uptake after chemotherapy correlated with good histological response and better outcome.[79-81]

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Patients with poor response to presurgical chemotherapy have an increased risk of local recurrence.[82]

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A retrospective analysis of risk factors for recurrence was performed in patients who received initial chemotherapy and underwent surgical resection of the primary tumor.[83][Level of evidence C1] Among 982 patients with a median follow-up of 7.6 years, the following was reported:

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Adverse risk factors for local recurrence were pelvic primary tumors (hazard ratio [HR], 2.04; 95% CI, 1.10–3.80) and marginal/intralesional resection (HR, 2.28; 95% CI, 1.25–4.16). The addition of radiation therapy was associated with improved outcome (HR, 0.52; 95% CI, 0.28–0.95).

Adverse risk factors for developing new pulmonary metastasis were less than 90% necrosis (HR, 2.13; 95% CI, 1.13–4.00) and previous pulmonary metastasis (HR, 4.90; 95% CI, 2.28–8.52).

Adverse risk factors for death included pulmonary metastasis (HR, 8.08; 95% CI, 4.01–16.29), bone or other metastasis (HR, 10.23; 95% CI, 4.90–21.36), and less than 90% necrosis (HR, 6.35; 95% CI, 3.18–12.69).

Early local recurrence (0–24 months) negatively influenced survival (HR, 3.79; 95% CI, 1.34–10.76).

In a retrospective cohort of 148 patients with pulmonary metastatic Ewing sarcoma, 41.2% had radiographic resolution of lung nodules after initial induction chemotherapy.[84] These patients had superior OS compared with patients who had residual nodules at end-induction (71.2% vs. 50.2% at 5 years). Particularly favorable outcomes were seen in the patients who had early clearance of lung nodules and received consolidative whole-lung radiation therapy (5-year OS rate, 85.2%).

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