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.
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BACKGROUND: 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. PATIENTS AND METHODS: 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. RESULTS: 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). CONCLUSION: 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.