Skip to main content
Top
Published in: Journal of Cancer Research and Clinical Oncology 4/2020

01-04-2020 | Metastasis | Original Article – Clinical Oncology

Synovial sarcoma disease characteristics and primary tumor sites differ between patient age groups: a report of the Cooperative Weichteilsarkom Studiengruppe (CWS)

Authors: Monika Scheer, Bernd Blank, Sebastian Bauer, Christian Vokuhl, Sabine Stegmaier, Simone Feuchtgruber, Anton Henssen, Monika Sparber-Sauer, Angelika Eggert, Ruppert Handgretinger, Arnulf Pekrun, Claudia Rossig, Stefan Rutkowski, Paul-Gerhardt Schlegel, Martin Schrappe, Thorsten Simon, Bernarda Kazanowska, Felix Niggli, Ruth Ladenstein, Gustaf Ljungman, Kirsi Jahnukainen, Jörg Fuchs, Stefan S. Bielack, Ewa Koscielniak, Thomas Klingebiel, The Cooperative Weichteilsarkom Studiengruppe [CWS]

Published in: Journal of Cancer Research and Clinical Oncology | Issue 4/2020

Login to get access

Abstract

Background

Older age is associated with worse outcome in synovial sarcoma (SS) patients. Differences in disease presentation among distinct age groups, however, are currently unknown.

Methods

SS patients < 21 years registered in consecutive CWS trials over the period of 1981–2018 were evaluated. Characteristics were analyzed according to age groups using the Fisher’s exact test.

Results

The study population included 432 SS patients. Disease characteristics differed according to age groups of children (0–12 years, n = 176), adolescents (13–16 years, n = 178), and young adults (17–21 years, n = 78). The proportion of invasive tumors (T2) was significantly higher in older patients: children 33%, adolescents 39% and young adults 54%, p = 0.009805. Similarly, the proportion of tumors > 10 cm was higher (13%, 21%, 31%; p = 0.005657) whereas conversely, the proportion of small tumors < 3 cm was lower in older patients (29%, 24%, 6%; p = 0.000104). The presence of metastases at first diagnosis was also highest in older patients (6%, 10%, 21%, p = 0.000963).
Notably, the proportion of thigh tumors was higher in older patients (p = 0.04173), whereas the proportion of head–neck tumors was lower in older patients (p = 0.08896).

Conclusions

The rates of large, invasive tumors and the presence of metastases are significantly associated with older patient age. Localization to the thigh is more frequent in older patients.

Discussion

The causes for these variations require further exploration.
Literature
go back to reference Bianchi G, Sambri A, Righi A et al (2017) Histology and grading are important prognostic factors in synovial sarcoma. Eur J Surg Oncol 43(9):1733–1739PubMed Bianchi G, Sambri A, Righi A et al (2017) Histology and grading are important prognostic factors in synovial sarcoma. Eur J Surg Oncol 43(9):1733–1739PubMed
go back to reference Bochennek K, Dantonello T, Koscielniak E et al (2013) Response of children with stage IV soft tissue sarcoma to topotecan and carboplatin: a phase II window trial of the cooperative soft tissue sarcoma group. Klin Padiatr 225(6):309–314PubMed Bochennek K, Dantonello T, Koscielniak E et al (2013) Response of children with stage IV soft tissue sarcoma to topotecan and carboplatin: a phase II window trial of the cooperative soft tissue sarcoma group. Klin Padiatr 225(6):309–314PubMed
go back to reference Brecht IB, Ferrari A, Int-Veen C et al (2006) Grossly-resected synovial sarcoma treated by the German and Italian pediatric soft tissue sarcoma cooperative groups: discussion on the role of adjuvant therapies. Pediatr Blood Cancer 46(1):11–17PubMed Brecht IB, Ferrari A, Int-Veen C et al (2006) Grossly-resected synovial sarcoma treated by the German and Italian pediatric soft tissue sarcoma cooperative groups: discussion on the role of adjuvant therapies. Pediatr Blood Cancer 46(1):11–17PubMed
go back to reference Brennan B, Stevens M, Kelsey A et al (2010) Synovial sarcoma in childhood and adolescence: a retrospective series of 77 patients registered by the Children's Cancer and Leukaemia Group between 1991 and 2006. Pediatr Blood Cancer 55(1):85–90PubMed Brennan B, Stevens M, Kelsey A et al (2010) Synovial sarcoma in childhood and adolescence: a retrospective series of 77 patients registered by the Children's Cancer and Leukaemia Group between 1991 and 2006. Pediatr Blood Cancer 55(1):85–90PubMed
go back to reference Brennan B, Stiller C, Grimer R et al (2016) Outcome and the effect of age and socioeconomic status in 1318 patients with synovial sarcoma in the english national cancer registry: 1985–2009. Clin Sarcoma Res 6:18PubMedPubMedCentral Brennan B, Stiller C, Grimer R et al (2016) Outcome and the effect of age and socioeconomic status in 1318 patients with synovial sarcoma in the english national cancer registry: 1985–2009. Clin Sarcoma Res 6:18PubMedPubMedCentral
go back to reference Chakiba C, Lagarde P, Pissaloux D et al (2014) Response to chemotherapy is not related to chromosome instability in synovial sarcoma. Ann Oncol 25(11):2267–2271PubMedPubMedCentral Chakiba C, Lagarde P, Pissaloux D et al (2014) Response to chemotherapy is not related to chromosome instability in synovial sarcoma. Ann Oncol 25(11):2267–2271PubMedPubMedCentral
go back to reference Dantonello TM, Int-Veen C, Winkler P et al (2008) Initial patient characteristics can predict pattern and risk of relapse in localized rhabdomyosarcoma. J Clin Oncol 26(3):406–413PubMed Dantonello TM, Int-Veen C, Winkler P et al (2008) Initial patient characteristics can predict pattern and risk of relapse in localized rhabdomyosarcoma. J Clin Oncol 26(3):406–413PubMed
go back to reference Dantonello TM, Int-Veen C, Harms D et al (2009) Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults. J Clin Oncol 27(9):1446–1455PubMed Dantonello TM, Int-Veen C, Harms D et al (2009) Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults. J Clin Oncol 27(9):1446–1455PubMed
go back to reference Ferrari A, Gronchi A, Casanova M et al (2004) Synovial sarcoma: a retrospective analysis of 271 patients of all ages treated at a single institution. Cancer 101(3):627–634PubMed Ferrari A, Gronchi A, Casanova M et al (2004) Synovial sarcoma: a retrospective analysis of 271 patients of all ages treated at a single institution. Cancer 101(3):627–634PubMed
go back to reference Ferrari A, De Salvo GL, Brennan B et al (2015) Synovial sarcoma in children and adolescents: the European pediatric soft tissue sarcoma study group prospective trial (EpSSG NRSTS 2005). Ann Oncol 26(3):567–572PubMed Ferrari A, De Salvo GL, Brennan B et al (2015) Synovial sarcoma in children and adolescents: the European pediatric soft tissue sarcoma study group prospective trial (EpSSG NRSTS 2005). Ann Oncol 26(3):567–572PubMed
go back to reference Ferrari A, Chi YY, De Salvo GL et al (2017) Surgery alone is sufficient therapy for children and adolescents with low-risk synovial sarcoma: a joint analysis from the European paediatric soft tissue sarcoma study group and the children's oncology group. Eur J Cancer 78:1–6PubMedPubMedCentral Ferrari A, Chi YY, De Salvo GL et al (2017) Surgery alone is sufficient therapy for children and adolescents with low-risk synovial sarcoma: a joint analysis from the European paediatric soft tissue sarcoma study group and the children's oncology group. Eur J Cancer 78:1–6PubMedPubMedCentral
go back to reference Gronchi A, Ferrari S, Quagliuolo V et al (2017) Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomised, controlled, phase 3, multicentre trial. Lancet Oncol 18(6):812–822PubMed Gronchi A, Ferrari S, Quagliuolo V et al (2017) Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomised, controlled, phase 3, multicentre trial. Lancet Oncol 18(6):812–822PubMed
go back to reference Guillou L, Benhattar J, Bonichon F et al (2004) Histologic grade, but not SYT-SSX fusion type, is an important prognostic factor in patients with synovial sarcoma: a multicenter, retrospective analysis. J Clin Oncol 22(20):4040–4050PubMed Guillou L, Benhattar J, Bonichon F et al (2004) Histologic grade, but not SYT-SSX fusion type, is an important prognostic factor in patients with synovial sarcoma: a multicenter, retrospective analysis. J Clin Oncol 22(20):4040–4050PubMed
go back to reference Harmer M, Denoix P, Hamperl H (1970) The TNM-system. Aktuelle Probl Chir 14:25–36PubMed Harmer M, Denoix P, Hamperl H (1970) The TNM-system. Aktuelle Probl Chir 14:25–36PubMed
go back to reference Kawai A, Woodruff J, Healey JH et al (1998) SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med 338(3):153–160PubMed Kawai A, Woodruff J, Healey JH et al (1998) SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med 338(3):153–160PubMed
go back to reference Koscielniak E, Jurgens H, Winkler K, et al. (1992) Treatment of soft tissue sarcoma in childhood and adolescence. A report of the German cooperative soft tissue sarcoma study. Cancer 70(10), 2557–2567 Koscielniak E, Jurgens H, Winkler K, et al. (1992) Treatment of soft tissue sarcoma in childhood and adolescence. A report of the German cooperative soft tissue sarcoma study. Cancer 70(10), 2557–2567
go back to reference Koscielniak E, Harms D, Henze G et al (1999) Results of treatment for soft tissue sarcoma in childhood and adolescence: a final report of the German cooperative soft tissue sarcoma study CWS-86. J Clin Oncol 17(12):3706–3719PubMed Koscielniak E, Harms D, Henze G et al (1999) Results of treatment for soft tissue sarcoma in childhood and adolescence: a final report of the German cooperative soft tissue sarcoma study CWS-86. J Clin Oncol 17(12):3706–3719PubMed
go back to reference Koscielniak E, Kosztyla D, Dantonello T et al (2013) Report of the CWS-2002-P study: treatment results for soft tissue sarcomas (STS) in childhood and adolescence. Pediatr Blood Cancer 60(S3):32 Koscielniak E, Kosztyla D, Dantonello T et al (2013) Report of the CWS-2002-P study: treatment results for soft tissue sarcomas (STS) in childhood and adolescence. Pediatr Blood Cancer 60(S3):32
go back to reference Ladanyi M (2005) Correlates of SYT-SSX fusion type in synovial sarcoma: getting more complex but also more interesting? Journal of clinical oncology:23(15):3638–3639 (author reply 3639-3640) Ladanyi M (2005) Correlates of SYT-SSX fusion type in synovial sarcoma: getting more complex but also more interesting? Journal of clinical oncology:23(15):3638–3639 (author reply 3639-3640)
go back to reference Ladanyi M, Antonescu CR, Leung DH et al (2002) Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients. Cancer Res 62(1):135–140PubMed Ladanyi M, Antonescu CR, Leung DH et al (2002) Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients. Cancer Res 62(1):135–140PubMed
go back to reference Lagarde P, Przybyl J, Brulard C et al (2013) Chromosome instability accounts for reverse metastatic outcomes of pediatric and adult synovial sarcomas. J Clin Oncol 31(5):608–615PubMed Lagarde P, Przybyl J, Brulard C et al (2013) Chromosome instability accounts for reverse metastatic outcomes of pediatric and adult synovial sarcomas. J Clin Oncol 31(5):608–615PubMed
go back to reference Lewis JJ, Antonescu CR, Leung DH et al (2000) Synovial sarcoma: a multivariate analysis of prognostic factors in 112 patients with primary localized tumors of the extremity. J Clin Oncol 18(10):2087–2094PubMed Lewis JJ, Antonescu CR, Leung DH et al (2000) Synovial sarcoma: a multivariate analysis of prognostic factors in 112 patients with primary localized tumors of the extremity. J Clin Oncol 18(10):2087–2094PubMed
go back to reference Mallen-St Clair J, Arshi A, Abemayor E et al (2016) Factors associated with survival in patients with synovial cell sarcoma of the head and neck: an analysis of 167 cases using the SEER (surveillance, epidemiology, and end results) database. JAMA Otolaryngol Head Neck Surg 142(6):576–583PubMed Mallen-St Clair J, Arshi A, Abemayor E et al (2016) Factors associated with survival in patients with synovial cell sarcoma of the head and neck: an analysis of 167 cases using the SEER (surveillance, epidemiology, and end results) database. JAMA Otolaryngol Head Neck Surg 142(6):576–583PubMed
go back to reference Maurer HM, Beltangady M, Gehan EA et al (1988) The intergroup rhabdomyosarcoma study-I A final report. Cancer 61(2):209–220PubMed Maurer HM, Beltangady M, Gehan EA et al (1988) The intergroup rhabdomyosarcoma study-I A final report. Cancer 61(2):209–220PubMed
go back to reference Modritz D, Ladenstein R, Potschger U et al (2005) Treatment for soft tissue sarcoma in childhood and adolescence. Austrian results within the CWS 96 study. Wien Klin Wochenschr 117(5–6):196–209PubMed Modritz D, Ladenstein R, Potschger U et al (2005) Treatment for soft tissue sarcoma in childhood and adolescence. Austrian results within the CWS 96 study. Wien Klin Wochenschr 117(5–6):196–209PubMed
go back to reference Okcu MF, Munsell M, Treuner J et al (2003) Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome. J Clin Oncol 21(8):1602–1611PubMed Okcu MF, Munsell M, Treuner J et al (2003) Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome. J Clin Oncol 21(8):1602–1611PubMed
go back to reference Orbach D, Mosseri V, Pissaloux D et al (2018) Genomic complexity in pediatric synovial sarcomas (Synobio study): the European pediatric soft tissue sarcoma group (EpSSG) experience. Cancer Med 7(4):1384–1393PubMedPubMedCentral Orbach D, Mosseri V, Pissaloux D et al (2018) Genomic complexity in pediatric synovial sarcomas (Synobio study): the European pediatric soft tissue sarcoma group (EpSSG) experience. Cancer Med 7(4):1384–1393PubMedPubMedCentral
go back to reference Outani H, Nakamura T, Murata H et al (2019) Localized synovial sarcoma: A single institutional study of 191 patients with a minimum follow-up of 5 years for survivors. J Surg Oncol 119(7):850–855PubMed Outani H, Nakamura T, Murata H et al (2019) Localized synovial sarcoma: A single institutional study of 191 patients with a minimum follow-up of 5 years for survivors. J Surg Oncol 119(7):850–855PubMed
go back to reference Palmerini E, Staals EL, Alberghini M et al (2009) Synovial sarcoma: retrospective analysis of 250 patients treated at a single institution. Cancer 115(13):2988–2998PubMed Palmerini E, Staals EL, Alberghini M et al (2009) Synovial sarcoma: retrospective analysis of 250 patients treated at a single institution. Cancer 115(13):2988–2998PubMed
go back to reference Riedel RF, Jones RL, Italiano A et al (2018) Systemic anti-cancer therapy in synovial sarcoma: a systematic review. Cancers (Basel) 10(11):1–19 Riedel RF, Jones RL, Italiano A et al (2018) Systemic anti-cancer therapy in synovial sarcoma: a systematic review. Cancers (Basel) 10(11):1–19
go back to reference Rothermundt C, Fischer GF, Bauer S et al (2018) Pre- and postoperative chemotherapy in localized extremity soft tissue sarcoma: a European organization for research and treatment of cancer expert survey. Oncologist 23(4):461–467PubMed Rothermundt C, Fischer GF, Bauer S et al (2018) Pre- and postoperative chemotherapy in localized extremity soft tissue sarcoma: a European organization for research and treatment of cancer expert survey. Oncologist 23(4):461–467PubMed
go back to reference Scheer M, Dantonello T, Hallmen E et al (2016a) Primary metastatic synovial sarcoma: experience of the CWS study group. Pediatr Blood Cancer 63(7):1198–1206PubMed Scheer M, Dantonello T, Hallmen E et al (2016a) Primary metastatic synovial sarcoma: experience of the CWS study group. Pediatr Blood Cancer 63(7):1198–1206PubMed
go back to reference Scheer M, Dantonello T, Hallmen E et al (2016b) Synovial sarcoma recurrence in children and young adults. Ann Surg Oncol 23(Suppl 5):618–626PubMed Scheer M, Dantonello T, Hallmen E et al (2016b) Synovial sarcoma recurrence in children and young adults. Ann Surg Oncol 23(Suppl 5):618–626PubMed
go back to reference Scheer M, Greulich M, Loff S et al (2019) Localized synovial sarcoma of the foot or ankle: a series of 32 Cooperative Weichteilsarkom study group patients. J Surg Oncol 119(1):109–119PubMed Scheer M, Greulich M, Loff S et al (2019) Localized synovial sarcoma of the foot or ankle: a series of 32 Cooperative Weichteilsarkom study group patients. J Surg Oncol 119(1):109–119PubMed
go back to reference Singer S, Baldini EH, Demetri GD et al (1996) Synovial sarcoma: prognostic significance of tumor size, margin of resection, and mitotic activity for survival. J Clin Oncol 14(4):1201–1208PubMed Singer S, Baldini EH, Demetri GD et al (1996) Synovial sarcoma: prognostic significance of tumor size, margin of resection, and mitotic activity for survival. J Clin Oncol 14(4):1201–1208PubMed
go back to reference Smolle MA, Parry M, Jeys L et al (2019) Synovial sarcoma: Do children do better? Eur J Surg Oncol 45(2):254–260PubMed Smolle MA, Parry M, Jeys L et al (2019) Synovial sarcoma: Do children do better? Eur J Surg Oncol 45(2):254–260PubMed
go back to reference Stegmaier S, Leuschner I, Poremba C et al (2017) The prognostic impact of SYT-SSX fusion type and histological grade in pediatric patients with synovial sarcoma treated according to the CWS (Cooperative Weichteilsarkom studie) trials. Pediatr Blood Cancer 64(1):89–95PubMed Stegmaier S, Leuschner I, Poremba C et al (2017) The prognostic impact of SYT-SSX fusion type and histological grade in pediatric patients with synovial sarcoma treated according to the CWS (Cooperative Weichteilsarkom studie) trials. Pediatr Blood Cancer 64(1):89–95PubMed
go back to reference Sultan I, Rodriguez-Galindo C, Saab R et al (2009) Comparing children and adults with synovial sarcoma in the Surveillance, epidemiology, and end results program, 1983–2005: an analysis of 1268 patients. Cancer 115(15):3537–3547PubMed Sultan I, Rodriguez-Galindo C, Saab R et al (2009) Comparing children and adults with synovial sarcoma in the Surveillance, epidemiology, and end results program, 1983–2005: an analysis of 1268 patients. Cancer 115(15):3537–3547PubMed
go back to reference Trassard M, Le Doussal V, Hacene K et al (2001) Prognostic factors in localized primary synovial sarcoma: a multicenter study of 128 adult patients. J Clin Oncol 19(2):525–534PubMed Trassard M, Le Doussal V, Hacene K et al (2001) Prognostic factors in localized primary synovial sarcoma: a multicenter study of 128 adult patients. J Clin Oncol 19(2):525–534PubMed
go back to reference van der Graaf WT, Orbach D, Judson IR et al (2017) Soft tissue sarcomas in adolescents and young adults: a comparison with their paediatric and adult counterparts. Lancet Oncol 18(3):e166–e175PubMed van der Graaf WT, Orbach D, Judson IR et al (2017) Soft tissue sarcomas in adolescents and young adults: a comparison with their paediatric and adult counterparts. Lancet Oncol 18(3):e166–e175PubMed
go back to reference Vlenterie M, Ho VK, Kaal SE et al (2015) Age as an independent prognostic factor for survival of localised synovial sarcoma patients. Br J Cancer 113(11):1602–1606PubMedPubMedCentral Vlenterie M, Ho VK, Kaal SE et al (2015) Age as an independent prognostic factor for survival of localised synovial sarcoma patients. Br J Cancer 113(11):1602–1606PubMedPubMedCentral
go back to reference Weiss SW, Goldblum JR (eds) (2008) Enzinger and Weiss's Soft Tissue Tumors. Mosby Elsevier, Philadelphia Weiss SW, Goldblum JR (eds) (2008) Enzinger and Weiss's Soft Tissue Tumors. Mosby Elsevier, Philadelphia
go back to reference Worch J, Ranft A, DuBois SG et al (2018) Age dependency of primary tumor sites and metastases in patients with Ewing sarcoma. Pediatr Blood Cancer 65(9):e27251PubMed Worch J, Ranft A, DuBois SG et al (2018) Age dependency of primary tumor sites and metastases in patients with Ewing sarcoma. Pediatr Blood Cancer 65(9):e27251PubMed
Metadata
Title
Synovial sarcoma disease characteristics and primary tumor sites differ between patient age groups: a report of the Cooperative Weichteilsarkom Studiengruppe (CWS)
Authors
Monika Scheer
Bernd Blank
Sebastian Bauer
Christian Vokuhl
Sabine Stegmaier
Simone Feuchtgruber
Anton Henssen
Monika Sparber-Sauer
Angelika Eggert
Ruppert Handgretinger
Arnulf Pekrun
Claudia Rossig
Stefan Rutkowski
Paul-Gerhardt Schlegel
Martin Schrappe
Thorsten Simon
Bernarda Kazanowska
Felix Niggli
Ruth Ladenstein
Gustaf Ljungman
Kirsi Jahnukainen
Jörg Fuchs
Stefan S. Bielack
Ewa Koscielniak
Thomas Klingebiel
The Cooperative Weichteilsarkom Studiengruppe [CWS]
Publication date
01-04-2020
Publisher
Springer Berlin Heidelberg
Published in
Journal of Cancer Research and Clinical Oncology / Issue 4/2020
Print ISSN: 0171-5216
Electronic ISSN: 1432-1335
DOI
https://doi.org/10.1007/s00432-019-03121-9

Other articles of this Issue 4/2020

Journal of Cancer Research and Clinical Oncology 4/2020 Go to the issue