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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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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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  6. Christian Vokuhl MD
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  7. Ivo Leuschner 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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