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

  • Original Article – Clinical Oncology
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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.

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Acknowledgements

We are indebted to the personnel of all participating institutions and thank all patients, parents, and guardians for their willingness to participate in the CWS-trials. We thank Iris Veit-Friedrich and Erika Hallmen for their excellent data management and Mary Morgan, MD for review of the manuscript.

Funding

Supported by the German Childhood Cancer Foundation, Bonn (A2007/13DKS2009.08); German Cancer Aid, Bonn (CWS-86: no. M34/87/Tr1, CWS-91: no. M76/91/Tr2, CWS-96: T9/96/TrI, CWS-2002-P: 50-2721-Tr2); Federal Ministry of Research and Technology, Bonn (CWS-81) and the Förderkreis Krebskranke Kinder e.V. Stuttgart, Germany.

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Authors and Affiliations

  1. Klinikum Stuttgart, Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Kriegsbergstrasse 62, 70174, Stuttgart, Germany

    Monika Scheer, Bernd Blank, Sabine Stegmaier, Simone Feuchtgruber, Monika Sparber-Sauer, Stefan S. Bielack & Ewa Koscielniak

  2. Sarcoma Center, West German Cancer Center, University of Duisburg‐Essen, Essen, Germany

    Sebastian Bauer

  3. Kiel Paediatric Tumor Registry, Department of Paediatric Pathology, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany

    Christian Vokuhl

  4. Department of Pediatric Hematology and Oncology, Charité-Universitätsmedizin Berlin, Berlin, Germany

    Anton Henssen & Angelika Eggert

  5. Department of Pediatric Hematology and Oncology, University Children’s Hospital, University Hospital Tuebingen, Tuebingen, Germany

    Ruppert Handgretinger & Ewa Koscielniak

  6. Department of Pediatric Hematology and Oncology, Hospital Bremen-Mitte, Bremen, Germany

    Arnulf Pekrun

  7. Department of Pediatric Hematology and Oncology, University Children’s Hospital Muenster, Muenster, Germany

    Claudia Rossig & Stefan S. Bielack

  8. Pediatric Hematology and Oncology, University Hamburg‐Eppendorf, Hamburg, Germany

    Stefan Rutkowski

  9. University Children’s Hospital, Pediatric Oncology, Hematology, Stem Cell Transplantation, University of Wuerzburg, Wuerzburg, Germany

    Paul-Gerhardt Schlegel

  10. Department of Pediatrics, University Hospital Schleswig-Holstein, Campus Kiel, Kiel, Germany

    Martin Schrappe

  11. Department of Pediatric Oncology and Hematology, University Children’s Hospital of Cologne, Medical Faculty, Cologne, Germany

    Thorsten Simon

  12. Department of Pediatric Oncology, University of Wroclaw, Wrocław, Poland

    Bernarda Kazanowska

  13. Department of Pediatric Oncology, University of Zuerich, Zuerich, Switzerland

    Felix Niggli

  14. St. Anna Kinderspital and St. Anna Kinderkrebsforschung e.V., Vienna, Austria

    Ruth Ladenstein

  15. Department of Women’s and Children’s Health, Uppsala University, Uppsala, Sweden

    Gustaf Ljungman

  16. New Children’s Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

    Kirsi Jahnukainen

  17. Department of Pediatric Surgery and Urology, University Children’s Hospital, Tuebingen, Germany

    Jörg Fuchs

  18. Hospital for Children and Adolescents, Goethe-University, Frankfurt (Main), Germany

    Thomas Klingebiel

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  1. Monika Scheer
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  2. Bernd Blank
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  3. Sebastian Bauer
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  16. Thorsten Simon
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  19. Ruth Ladenstein
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Correspondence to Monika Scheer.

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Scheer, M., Blank, B., Bauer, S. et al. Synovial sarcoma disease characteristics and primary tumor sites differ between patient age groups: a report of the Cooperative Weichteilsarkom Studiengruppe (CWS). J Cancer Res Clin Oncol 146, 953–960 (2020). https://doi.org/10.1007/s00432-019-03121-9

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  • DOI: https://doi.org/10.1007/s00432-019-03121-9

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