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037 _ _ |a DKFZ-2025-02375
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a Heinz, Amadeus T
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245 _ _ |a Children and Adolescents With Localised Non-Rhabdomyosarcoma Soft Tissue Sarcoma: Results of the CWS-96 and CWS-2002P Prospective Trials With Reclassification of the Trial Data Incorporating the Recent Soft Tissue Sarcoma Registry.
260 _ _ |a New York, NY
|c 2025
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520 _ _ |a Here we report the results of the first comprehensive European trials for all soft tissue sarcoma entities, which aimed to develop a new risk stratification system, limit radiotherapy and chemotherapy for low-/standard-risk patients, and evaluate different chemotherapy regimens for high-risk patients.The prospective CWS-96 and CWS-2002P trials stratified patients with non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) by Intergroup Rhabdomyosarcoma Study (IRS) group, histology and grade (CWS-96) or IRS group, lymph node status, tumour histology and size (CWS-2002P). Low-risk patients received no additional treatment. Standard-risk patients were treated with 44.8 Gy radiotherapy, and in CWS-96, adjuvant chemotherapy was added for high-grade tumours (vincristine, actinomycin-D, ifosfamide, 160-240 mg/m2 adriamycin/VAIA). High-risk patients received VAIA in CWS-96 or an intensified adriamycin regimen in CWS-2002P (VAIA-III, 320 mg/m2 adriamycin), resection and/or 44.8 Gy radiotherapy, followed by maintenance treatment with cyclophosphamide and vinblastine in certain NRSTS in CWS-2002P. The trials enrolled 1249 patients with localised NRSTS, of whom 483 (CWS-96) and 445 (CWS-2002P) were eligible for survival analysis. Histological classifications were retrospectively standardised, including recent data from the soft tissue sarcoma registry.Overall survival (OS) was significantly improved (5-year OS: 81% vs. 73%, p = 0.024) for patients treated in CWS-2002P compared to CWS-96, partly explained by the inclusion of more low-grade entities in CWS-2002P. The higher anthracycline dose in the VAIA-III regimen (CWS-2002P) did not improve survival (EFS: p = 0.23, OS: p = 0.73; matched pairs analysis). Re-classification revealed a higher proportion of malignant entities according to WHO in CWS-96 compared to CWS-2002P (90% vs. 81%, p < 0.001).Complete resection alone is sufficient to cure low-risk NRSTS. Survival was not improved by dose-intensified adjuvant chemotherapy (with/without maintenance treatment). Patients with completely resected high-risk disease may not profit from adjuvant radiotherapy.
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650 _ 7 |a CWS‐2002P
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650 _ 7 |a CWS‐96
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650 _ 7 |a anthracyclines
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650 _ 7 |a chemotherapy
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650 _ 7 |a long‐term sequelae
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650 _ 7 |a maintenance treatment
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650 _ 7 |a non‐rhabdomyosarcoma soft tissue sarcoma
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650 _ 7 |a pediatric/adolescent oncology
|2 Other
650 _ 7 |a radiotherapy
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650 _ 7 |a surgery
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700 1 _ |a Schönstein, Anton
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700 1 _ |a Koscielniak, Ewa
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700 1 _ |a Ebinger, Martin
|0 0000-0002-4229-8058
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700 1 _ |a Fuchs, Jörg
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700 1 _ |a Harrabi, Semi
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700 1 _ |a Vokuhl, Christian
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700 1 _ |a Imle, Roland
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700 1 _ |a Stegmaier, Sabine
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700 1 _ |a Brecht, Ines B
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700 1 _ |a Ljungman, Gustaf
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700 1 _ |a Juntti, Hanna
|b 11
700 1 _ |a Ladenstein, Ruth
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700 1 _ |a Kazanowska, Bernarda
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700 1 _ |a Klingebiel, Thomas
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700 1 _ |a Pajtler, Kristian
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700 1 _ |a Sparber-Sauer, Monika
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