TY  - JOUR
A1  - Scheer, Monika
A1  - Vokuhl, Christian Oliver
A1  - Bauer, Sebastian
A1  - Fuchs, Jörg
A1  - Loff, Steffan
A1  - Timmermann, Beate
A1  - Münter, Marc
A1  - Henssen, Anton George
A1  - Kazanowska, Bernarda
A1  - Niggli, Felix
A1  - Ladenstein, Ruth
A1  - Ljungman, Gustaf
A1  - Koscielniak, Ewa
A1  - Klingebiel, Thomas
T1  - The effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents
T2  - Journal of cancer research and clinical oncology
N2  - 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.
KW  - Synovial sarcoma
KW  - Soft-tissue sarcoma
KW  - Pediatric sarcoma
KW  - Adjuvant therapies
KW  - Chemotherapy
KW  - Radiotherapy
Y1  - 2021
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/69516
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-695165
SN  - 1432-1335
N1  - Open Access funding enabled and organized by Projekt DEAL. Supported by the German Childhood Cancer Foundation, Bonn; German Cancer Aid, Bonn; Federal Ministry of Research and Technology, Bonn and the Förderkreis Krebskranke Kinder e.V. Stuttgart, Germany.
VL  - 147
IS  - 12
SP  - 3735
EP  - 3747
PB  - Springer
CY  - Berlin ; Heidelberg
ER  -