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The effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents

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Item Type:Article
Title:The effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents
Creators Name:Scheer, M., Vokuhl, C., Bauer, S., Fuchs, J., Loff, S., Timmermann, B., Münter, M., Henssen, A.G., Kazanowska, B., Niggli, F., Ladenstein, R., Ljungman, G., Koscielniak, E. and Klingebiel, T.
Abstract:BACKGROUND: The benefit of adjuvant therapy in synovial sarcoma (SS) treatment is under debate. Long-term follow-up data are missing. METHODS: SS patients treated in the consecutive trials CWS-81, CWS-86, CWS-91, CWS-96, CWS-2002-P, and the SoTiSaR-registry till 2013 were analyzed. RESULTS: Median age of 185 patients was 13.9 years (0.1-56)-with median follow-up of 7.4 years for 163 survivors. Most tumors (76%) were located in extremities. Size was < 3 cm in 58 (31%), 3-5 cm in 59 (32%), 5-10 cm in 42 (23%), and > 10 cm in 13 (7%) (13 missing). In 84 (45%) tumors, first excision was complete (R0 corresponding to IRS-I-group) and in 101 (55%) marginal (R1 corresponding to IRS-II-group). In a subsequent surgical intervention during chemotherapy, R0-status was accomplished in 23 additional IRS-II-group patients with secondary surgery. Radiotherapy was administered to 135 (73%), thereof 62 with R0-status and 67 R1-status (6 missing information). Adjuvant chemotherapy was administered to all but six patients. 5-year event-free (EFS) and overall survival (OS) was 82.9% ± 5.7 (95%CI) and 92.5% ± 3.9. Local and metastatic relapse-free survival was 91.3% ± 4.3 and 92.3% ± 4.1 at 5 years, respectively. In the multivariate analysis, tumor size and no chemotherapy were independently associated with EFS. Size and site were associated with OS. In a detailed analysis of local and metastatic events, tumor size was associated with an independent risk for developing metastases. No independent factor for suffering local recurrence could be identified. DISCUSSION: Omission of chemotherapy in a non-stratified way seems not justified. Size governs survival due to high linear association with risk of suffering metastatic recurrence in a granular classification.
Keywords:Synovial Sarcoma, Soft-Tissue Sarcoma, Pediatric Sarcoma, Adjuvant Therapies, Chemotherapy, Radiotherapy
Source:Journal of Cancer Research and Clinical Oncology
ISSN:0171-5216
Publisher:Springer
Volume:147
Number:12
Page Range:3735-3747
Date:December 2021
Official Publication:https://doi.org/10.1007/s00432-021-03614-6
PubMed:View item in PubMed

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