Cargando…
The effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents
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 a...
Autores principales: | , , , , , , , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2021
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8557198/ https://www.ncbi.nlm.nih.gov/pubmed/34272609 http://dx.doi.org/10.1007/s00432-021-03614-6 |
_version_ | 1784592326145867776 |
---|---|
author | Scheer, Monika Vokuhl, Christian Bauer, Sebastian Fuchs, Jörg Loff, Steffan Timmermann, Beate Münter, Marc Henssen, Anton George Kazanowska, Bernarda Niggli, Felix Ladenstein, Ruth Ljungman, Gustaf Koscielniak, Ewa Klingebiel, Thomas |
author_facet | Scheer, Monika Vokuhl, Christian Bauer, Sebastian Fuchs, Jörg Loff, Steffan Timmermann, Beate Münter, Marc Henssen, Anton George Kazanowska, Bernarda Niggli, Felix Ladenstein, Ruth Ljungman, Gustaf Koscielniak, Ewa Klingebiel, Thomas |
author_sort | Scheer, Monika |
collection | PubMed |
description | 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. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00432-021-03614-6. |
format | Online Article Text |
id | pubmed-8557198 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-85571982021-11-15 The effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents Scheer, Monika Vokuhl, Christian Bauer, Sebastian Fuchs, Jörg Loff, Steffan Timmermann, Beate Münter, Marc Henssen, Anton George Kazanowska, Bernarda Niggli, Felix Ladenstein, Ruth Ljungman, Gustaf Koscielniak, Ewa Klingebiel, Thomas J Cancer Res Clin Oncol Original Article – Clinical Oncology 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. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00432-021-03614-6. Springer Berlin Heidelberg 2021-07-17 2021 /pmc/articles/PMC8557198/ /pubmed/34272609 http://dx.doi.org/10.1007/s00432-021-03614-6 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . |
spellingShingle | Original Article – Clinical Oncology Scheer, Monika Vokuhl, Christian Bauer, Sebastian Fuchs, Jörg Loff, Steffan Timmermann, Beate Münter, Marc Henssen, Anton George Kazanowska, Bernarda Niggli, Felix Ladenstein, Ruth Ljungman, Gustaf Koscielniak, Ewa Klingebiel, Thomas The effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents |
title | The effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents |
title_full | The effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents |
title_fullStr | The effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents |
title_full_unstemmed | The effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents |
title_short | The effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents |
title_sort | effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents |
topic | Original Article – Clinical Oncology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8557198/ https://www.ncbi.nlm.nih.gov/pubmed/34272609 http://dx.doi.org/10.1007/s00432-021-03614-6 |
work_keys_str_mv | AT scheermonika theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT vokuhlchristian theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT bauersebastian theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT fuchsjorg theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT loffsteffan theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT timmermannbeate theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT muntermarc theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT henssenantongeorge theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT kazanowskabernarda theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT nigglifelix theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT ladensteinruth theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT ljungmangustaf theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT koscielniakewa theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT klingebielthomas theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT theeffectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT scheermonika effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT vokuhlchristian effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT bauersebastian effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT fuchsjorg effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT loffsteffan effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT timmermannbeate effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT muntermarc effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT henssenantongeorge effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT kazanowskabernarda effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT nigglifelix effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT ladensteinruth effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT ljungmangustaf effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT koscielniakewa effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT klingebielthomas effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents AT effectofadjuvanttherapiesonlongtermoutcomeforprimaryresectedsynovialsarcomainaseriesofmainlychildrenandadolescents |