Monika Scheer1, Tobias Dantonello2, Erika Hallmen2, Bernd Blank2, Monika Sparber-Sauer2, Christian Vokuhl3, Ivo Leuschner3, Marc W Münter4, Thekla von Kalle5, Stefan S Bielack2,6, Thomas Klingebiel7, Ewa Koscielniak2,8. 1. Pediatrics 5 (Oncology, Hematology, Immunology), Olgahospital, Klinikum Stuttgart, Stuttgart, Germany. monika.scheer@olgahospital-stuttgart.de. 2. Pediatrics 5 (Oncology, Hematology, Immunology), Olgahospital, Klinikum Stuttgart, Stuttgart, Germany. 3. Kiel Pediatric Tumor Registry, Department of Pediatric Pathology, University Hospital Kiel, Kiel, Germany. 4. Department of Radiation Oncology, Klinikum Stuttgart, Stuttgart, Germany. 5. Pediatric Radiology, Radiologisches Institut, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany. 6. Department of Pediatric Hematology and Oncology, University Children's Hospital, Münster, Germany. 7. Hospital for Children and Adolescents, Goethe-University Frankfurt, Frankfurt am Main, Germany. 8. Department of Pediatric Oncology, University of Tübingen, Tübingen, Germany.
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.
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.
Authors: Monika Scheer; Tobias Dantonello; Peter Brossart; Dagmar Dilloo; Lothar Schweigerer; Simone Feuchtgruber; Monika Sparber-Sauer; Christian Vokuhl; Stefan S Bielack; Thomas Klingebiel; Ewa Koscielniak; Thekla von Kalle Journal: Pediatr Radiol Date: 2018-01-24
Authors: Monika Scheer; Christian Vokuhl; Bernd Blank; Erika Hallmen; Thekla von Kalle; Marc Münter; Rüdiger Wessalowski; Maite Hartwig; Monika Sparber-Sauer; Paul-Gerhardt Schlegel; Christof M Kramm; Udo Kontny; Bernd Spriewald; Thomas Kegel; Sebastian Bauer; Bernarda Kazanowska; Felix Niggli; Ruth Ladenstein; Gustaf Ljungman; Kirsi Jahnukainen; Jörg Fuchs; Stefan S Bielack; Thomas Klingebiel; Ewa Koscielniak Journal: Cancer Med Date: 2019-01-16 Impact factor: 4.452
Authors: Richard F Riedel; Robin L Jones; Antoine Italiano; Chet Bohac; Juliette C Thompson; Kerstin Mueller; Zaeem Khan; Seth M Pollack; Brian A Van Tine Journal: Cancers (Basel) Date: 2018-11-01 Impact factor: 6.639