Primary Metastatic Synovial Sarcoma: Experience of the CWS Study Group
Corresponding Author
Monika Scheer MD
Pediatrics 5, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany
Correspondence to: Monika Scheer, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Olgahospital, Kriegsbergstrasse 62, D-70174 Stuttgart, Germany. E-mail: [email protected]; [email protected]
Search for more papers by this authorTobias Dantonello MD
Pediatrics 5, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany
Search for more papers by this authorErika Hallmen CDM
Pediatrics 5, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany
Search for more papers by this authorChristian Vokuhl MD
Department of Pediatric Pathology, Kiel Pediatric Tumor Registry, University Hospital of Schleswig-Holstein, Campus Kiel, Germany
Search for more papers by this authorIvo Leuschner MD
Department of Pediatric Pathology, Kiel Pediatric Tumor Registry, University Hospital of Schleswig-Holstein, Campus Kiel, Germany
Search for more papers by this authorMonika Sparber-Sauer MD
Pediatrics 5, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany
Search for more papers by this authorBernarda Kazanowska MD
Department of Pediatric Hematology/Oncology and BMT, University of Wroclaw, Wroclaw, Poland
Search for more papers by this authorFelix Niggli MD
Department of Pediatric Oncology, University of Zurich, Zurich, Switzerland
Search for more papers by this authorRuth Ladenstein MD
St. Anna Kinderspital and St. Anna Kinderkrebsforschung e.V, Vienna, Austria
Search for more papers by this authorStefan S. Bielack MD
Pediatrics 5, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany
Department of Pediatric Hematology and Oncology, University Children's Hospital Muenster, Münster, Germany
Search for more papers by this authorThomas Klingebiel MD
Hospital for Children and Adolescents, Goethe-University Frankfurt, Frankfurt am Main, Germany
Search for more papers by this authorEwa Koscielniak MD
Pediatrics 5, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany
Department of Pediatric Oncology, University of Tuebingen, Tuebingen, Germany
Search for more papers by this authorCorresponding Author
Monika Scheer MD
Pediatrics 5, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany
Correspondence to: Monika Scheer, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Olgahospital, Kriegsbergstrasse 62, D-70174 Stuttgart, Germany. E-mail: [email protected]; [email protected]
Search for more papers by this authorTobias Dantonello MD
Pediatrics 5, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany
Search for more papers by this authorErika Hallmen CDM
Pediatrics 5, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany
Search for more papers by this authorChristian Vokuhl MD
Department of Pediatric Pathology, Kiel Pediatric Tumor Registry, University Hospital of Schleswig-Holstein, Campus Kiel, Germany
Search for more papers by this authorIvo Leuschner MD
Department of Pediatric Pathology, Kiel Pediatric Tumor Registry, University Hospital of Schleswig-Holstein, Campus Kiel, Germany
Search for more papers by this authorMonika Sparber-Sauer MD
Pediatrics 5, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany
Search for more papers by this authorBernarda Kazanowska MD
Department of Pediatric Hematology/Oncology and BMT, University of Wroclaw, Wroclaw, Poland
Search for more papers by this authorFelix Niggli MD
Department of Pediatric Oncology, University of Zurich, Zurich, Switzerland
Search for more papers by this authorRuth Ladenstein MD
St. Anna Kinderspital and St. Anna Kinderkrebsforschung e.V, Vienna, Austria
Search for more papers by this authorStefan S. Bielack MD
Pediatrics 5, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany
Department of Pediatric Hematology and Oncology, University Children's Hospital Muenster, Münster, Germany
Search for more papers by this authorThomas Klingebiel MD
Hospital for Children and Adolescents, Goethe-University Frankfurt, Frankfurt am Main, Germany
Search for more papers by this authorEwa Koscielniak MD
Pediatrics 5, Klinikum Stuttgart, Olgahospital, Stuttgart, Germany
Department of Pediatric Oncology, University of Tuebingen, Tuebingen, Germany
Search for more papers by this authorGrant sponsor: German Childhood Cancer Foundation, Bonn; Grant sponsor: German Cancer Aid, Bonn; Grant sponsor: Federal Ministry of Research and Technology, Bonn; Grant sponsor: Förderkreis Krebskranke Kinder e.V. Stuttgart, Germany.
Conflict of interest: Nothing to declare
Abstract
Background
Prognostic factors for localized synovial sarcoma are well defined. However, few data exist regarding patients with metastases at diagnosis. Poor outcome is described but the optimal therapeutic regimen remains unclear. Our aim was to assess the outcome, identify prognostic factors, and analyze treatment strategies.
Methods
Patients <21 years with synovial sarcoma and primary distant metastases treated in the consecutive prospective European Cooperative Weichteilsarkom Studiengruppe trials 1980–2010 were analyzed.
Results
Twenty-nine of 296 patients had primary metastases. Twenty-seven could be included. Median age was 16.7 years. Primaries were mainly located in the limbs (78%) and 74% were ≥10 cm. Metastases involved the lungs in all patients. Two patients presented with synchronous bone metastases. Sixty-three percent of patients achieved a first remission, whereas only 26% maintained it. Relapses were metastatic with pulmonary metastases in nearly all patients. Five-year event-free survival and overall survival (OS) rates were 26% and 30%, respectively. Prognosis was best for patients with oligometastatic lung metastases (5-year OS probability 85%). Prognosis was worse for patients with multiple bilateral lung metastases (5-year OS 13%) and even poorer for those with concurrent bone metastases. Treatment elements associated with superior survival were adequate local therapy of the primary tumor and, if feasible, for metastases, chemotherapy with an ifosfamide/doxorubicin-based regimen. The use of whole lung irradiation was not correlated with better outcomes.
Conclusions
The overall prognosis of primary metastatic synovial sarcoma is poor. However, individuals with oligometastatic lung metastases had very good chance for long-term survival when treated with adequate multimodal therapy.
Supporting Information
Additional supporting information can be found in the supporting information tab for this article.
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