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Synovial Sarcoma Recurrence in Children and Young Adults

  • Bone and Soft Tissue Sarcomas
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Recurrence of synovial sarcoma (SS) has been associated with poor prognosis. Optimal treatment is unknown due to heterogeneous primary therapies with or without chemotherapy.

Methods

Data of patients treated in consecutive prospective European Cooperative Weichteilsarkom Studiengruppe trials 1981–2010 with primary localized SS less than 21 years were analyzed. Chemotherapy had been recommended for all SS patients during primary therapy.

Results

Of 220 patients, 52 experienced recurrence a median of 2.5 years (range, 0.3–11.6 years) after their initial diagnosis. Recurrence was local in 22 (42 %), metastatic in 24 (46 %), and combined in 6 (12 %) of the 52 patients. If present, metastases involved the lungs in more than 90 % of the patients. Second remission was achieved by 39 (75 %) of the 52 patients, whereas only 12 (23 %) of the 39 patients maintained it. The median follow-up period for 17 survivors was 6.7 years (range, 3.2–19.6 years). The 5-year post-relapse event-free survival probability was 26 %, and the overall survival probability was 40 %. In the univariable analyses, initial tumor smaller than 3 cm, 2.5 years or longer to recurrence, local relapse only, and R0/R1 resection at relapse correlated with improved survival expectancies. In the multivariable analysis, the only factor retaining significance was R0/R1 resection of the recurrence. No difference between R0 and R1 resections was evident. For the patients with metastatic relapse, maintenance therapy seemed to prolong the time to subsequent recurrences.

Conclusion

Although 75 % of the patients with first SS recurrence achieved a second remission, only a minority became long-term, disease-free survivors. They had small tumors at initial diagnosis, local relapse as the only site of involvement, and complete resection of their recurrence. Because the majority of patients relapse subsequently, quality-of-life-based treatment approaches prolonging disease-free intervals are needed.

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References

  1. Weiss SW, Goldblum JR (eds). Enzinger and Weiss’s Soft Tissue Tumors. Mosby Elsevier, Philadelphia, 2008.

    Google Scholar 

  2. Okcu MF, Munsell M, Treuner J, et al. Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome. J Clin Oncol. 2003:21:1602–11.

    Article  PubMed  Google Scholar 

  3. Ferrari A, De Salvo GL, Brennan B, et al. Synovial sarcoma in children and adolescents: the European Pediatric Soft Tissue Sarcoma Study Group prospective trial (EpSSG NRSTS 2005). Ann Oncol. 2015:26:567–72.

    Article  CAS  PubMed  Google Scholar 

  4. Kawai A, Woodruff J, Healey JH, et al. SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med. 1998:338:153–60.

    Article  CAS  PubMed  Google Scholar 

  5. Ladanyi M. Correlates of SYT-SSX fusion type in synovial sarcoma: getting more complex but also more interesting? J Clin Oncol. 2005:23:3638–9; author reply 3639–40.

  6. Fisher C. Synovial sarcoma. Ann Diagn Pathol. 1998:2:401–21.

    Article  CAS  PubMed  Google Scholar 

  7. Ferrari A, Bisogno G, Alaggio R, et al. Synovial sarcoma of children and adolescents: the prognostic role of axial sites. Eur J Cancer. 2008:44:1202–9.

    Article  PubMed  Google Scholar 

  8. Weihkopf T, Blettner M, Dantonello T, et al. Incidence and time trends of soft tissue sarcomas in German children 1985–2004: a report from the population-based German Childhood Cancer Registry. Eur J Cancer. 2008:44:432–40.

    Article  PubMed  Google Scholar 

  9. Sultan I, Rodriguez-Galindo C, Saab R, et al. Comparing children and adults with synovial sarcoma in the Surveillance, Epidemiology, and End Results program, 1983 to 2005: an analysis of 1268 patients. Cancer. 2009:115:3537–47.

    Article  PubMed  Google Scholar 

  10. Ferrari A, De Salvo GL, Dall’Igna P, et al. Salvage rates and prognostic factors after relapse in children and adolescents with initially localised synovial sarcoma. Eur J Cancer. 2012:48:3448–55.

    Article  PubMed  Google Scholar 

  11. Soole F, Maupain C, Defachelles AS, et al. Synovial sarcoma relapses in children and adolescents: prognostic factors, treatment, and outcome. Pediatr Blood Cancer. 2014:61:1387–93.

    Article  PubMed  Google Scholar 

  12. Koscielniak E, Jurgens H, Winkler K, et al. Treatment of soft tissue sarcoma in childhood and adolescence: a report of the German Cooperative Soft Tissue Sarcoma Study. Cancer. 1992:70:2557–67.

    Article  CAS  PubMed  Google Scholar 

  13. Koscielniak E, Harms D, Henze G, et al. 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. 1999:17:3706–19.

    CAS  PubMed  Google Scholar 

  14. Dantonello TM, Int-Veen C, Harms D, et al. Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults. J Clin Oncol. 2009:27:1446–55.

    Article  CAS  PubMed  Google Scholar 

  15. Modritz D, Ladenstein R, Potschger U, et al. Treatment for soft tissue sarcoma in childhood and adolescence: Austrian results within the CWS 96 study. Wien Klin Wochenschr. 2005:117:196–209.

    Article  PubMed  Google Scholar 

  16. Bochennek K, Dantonello T, Koscielniak E, et al. 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. 2013:225:309–14.

    Article  CAS  PubMed  Google Scholar 

  17. Koscielniak E, Kosztyla D, Dantonello T, et al. Report of the CWS-2002-P study: treatment results for soft tissue sarcomas (STS) in childhood and adolescence. Pediatr Blood Cancer. 2013:60:32.

    Article  Google Scholar 

  18. Dantonello TM, Int-Veen C, Winkler P, et al. Initial patient characteristics can predict pattern and risk of relapse in localized rhabdomyosarcoma. J Clin Oncol. 2008:26:406–13.

    Article  PubMed  Google Scholar 

  19. Klingebiel T, Pertl U, Hess CF, et al. Treatment of children with relapsed soft tissue sarcoma: report of the German CESS/CWS REZ 91 trial. Med Pediatr Oncol. 1998:30:269–75.

    Article  CAS  PubMed  Google Scholar 

  20. Klingebiel T, Boos J, Beske F, et al. Treatment of children with metastatic soft tissue sarcoma with oral maintenance compared to high-dose chemotherapy: report of the HD CWS-96 trial. Pediatr Blood Cancer. 2008:50:739–45.

    Article  PubMed  Google Scholar 

  21. Siepermann M, Koscielniak E, Dantonello T, et al. Oral low-dose chemotherapy: successful treatment of an alveolar rhabdomyosarcoma during pregnancy. Pediatr Blood Cancer. 2012:58:104–6.

    Article  PubMed  Google Scholar 

  22. Scheer M, Dantonello T, Hallmen E, et al. Primary metastatic synovial sarcoma: experience of the CWS Study Group. Pediatr Blood Cancer. 2016:63:1198–206.

    Article  CAS  PubMed  Google Scholar 

  23. Kaplan EL, Meyer P. Nonparametric estimation from incomplete observations. J Am Stat Assoc. 1958:53:457–81.

    Article  Google Scholar 

  24. Greenwood M. Reports on Public Health and Medical Subjects: The Errors of Sampling of the Survivorship Tables. HM Stationery Office, London, 1926.

    Google Scholar 

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Acknowledgment

We are indebted to the personnel of all the participating institutions and thank all patients, parents, and guardians for their willingness to participate in the CWS trials. We thank Iris Veit-Friedrich and Simone Feuchtgruber for their excellent data management. This study was supported by the German Childhood Cancer Foundation, Bonn; German Cancer Aid, Bonn; the Federal Ministry of Research and Technology, Bonn; and the Förderkreis Krebskranke Kinder e.V. Stuttgart, Germany.

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Authors and Affiliations

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

    Monika Scheer MD, Tobias Dantonello MD, Erika Hallmen CMD, Bernd Blank MD, Monika Sparber-Sauer MD, Stefan S. Bielack MD & Ewa Koscielniak MD

  2. Kiel Pediatric Tumor Registry, Department of Pediatric Pathology, University Hospital Kiel, Kiel, Germany

    Christian Vokuhl MD & Ivo Leuschner MD

  3. Department of Radiation Oncology, Klinikum Stuttgart, Stuttgart, Germany

    Marc W. Münter MD

  4. Pediatric Radiology, Radiologisches Institut, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany

    Thekla von Kalle MD

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

    Stefan S. Bielack MD

  6. Hospital for Children and Adolescents, Goethe-University Frankfurt, Frankfurt am Main, Germany

    Thomas Klingebiel MD

  7. Department of Pediatric Oncology, University of Tübingen, Tübingen, Germany

    Ewa Koscielniak MD

Authors
  1. Monika Scheer MD
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  2. Tobias Dantonello MD
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  3. Erika Hallmen CMD
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  4. Bernd Blank MD
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  5. Monika Sparber-Sauer MD
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  8. Marc W. Münter MD
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  9. Thekla von Kalle MD
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  10. Stefan S. Bielack MD
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  11. Thomas Klingebiel MD
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  12. Ewa Koscielniak MD
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Consortia

On behalf of the Cooperative Weichteilsarkom Studiengruppe [CWS]

Corresponding author

Correspondence to Monika Scheer MD.

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Scheer, M., Dantonello, T., Hallmen, E. et al. Synovial Sarcoma Recurrence in Children and Young Adults. Ann Surg Oncol 23 (Suppl 5), 618–626 (2016). https://doi.org/10.1245/s10434-016-5535-2

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  • DOI: https://doi.org/10.1245/s10434-016-5535-2

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