Jörg Fuchs1, Cristian Urla2, Monika Sparber-Sauer3, Andreas Schuck4, Ivo Leuschner5, Thomas Klingebiel6, Gunnar Blumenstock7, Guido Seitz8, Ewa Koscielniak3. 1. Department of Pediatric Surgery and Pediatric Urology, University Children's Hospital of Tuebingen, Hoppe-Seyler-Str. 3, 72076, Tuebingen, Germany. Joerg.Fuchs@med.uni-tuebingen.de. 2. Department of Pediatric Surgery and Pediatric Urology, University Children's Hospital of Tuebingen, Hoppe-Seyler-Str. 3, 72076, Tuebingen, Germany. 3. Klinikum Stuttgart, Zentrum für Kinder-, Jugend-und Frauenmedizin, Olgahospital, Pediatrics 5 (Pediatric Oncology Hematology and Immunology), Kriegsbergstrasse 62, 70174, Stuttgart, Germany. 4. Department of Radiotherapy, University Hospital Münster, Germany, Albert-Schweitzer-Str. 33, 48143, Münster, Germany. 5. Department of Pediatric Pathology, University Hospital Schleswig-Holstein, Arnold-Heller-Str. 3/14, 24105, Kiel, Germany. 6. Department of Pediatric Hematology and Oncology, University Hospital Frankfurt, Theodor-Stern-Kai 7, 60590, Frankfurt/main, Germany. 7. Department of Clinical Epidemiology and Applied Biometry, University Hospital of Tuebingen, Silcherstraße 5, 72076, Tübingen, Germany. 8. Department of Pediatric Surgery,Department of Pediatric Surgery and Pediatric, University Hospital Marburg, 35043, Baldingerstraße, Marburg, Germany.
Abstract
PURPOSE: Rhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma. In 7% of the cases it is localized at the chest wall or intrathoracically. The aim of this study was to analyze the multimodal treatment concepts and outcomes of children suffering from intrathoracic and chest wall RMS treated within three different Cooperative Soft Tissue Sarcoma (CWS) trials and one registry (Soft Tissue Sarcoma Registry, SoTiSaR). METHODS: Data of 51 patients with thoracic RMS enrolled in three different CWS trials (CWS-86, -91, -2002P) and one registry (SoTiSaR) were analyzed retrospectively. Surgery and its influence on outcome were assessed. Median follow-up was 37.5 months (0.9-152.5). RESULTS: Median age of the patients was 8.8 years (range 0-19 years). The 5-year overall survival rate (OS) was 57% (95%-CI 49-65) and the 5-year event-free survival rate (EFS) was 45% (38-52). Thirty-five patients had tumors located at the chest wall (EFS: 51%, 43-59), and 16 patients had intrathoracic tumors (EFS: 26%, 13-39). Seventeen patients with tumors ≤ 5 cm had a better outcome (EFS: 64%, 52-76) compared to patients with tumors larger than 5 cm (EFS: 36%, 27-45). Radiotherapy (RT) significantly improved the survival of patients with alveolar RMS compared to patients with embryonal histology (EFS: 66%, 52-80 vs. 32%, 21-43 p = 0.02). Complete tumor excision during delayed surgery was the main prognostic factor for survival (p = 0.045). CONCLUSION: Thoracic RMS is a rare tumor entity. Completeness of tumor resection significantly improved survival of the patients.
PURPOSE:Rhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma. In 7% of the cases it is localized at the chest wall or intrathoracically. The aim of this study was to analyze the multimodal treatment concepts and outcomes of children suffering from intrathoracic and chest wall RMS treated within three different Cooperative Soft Tissue Sarcoma (CWS) trials and one registry (Soft Tissue Sarcoma Registry, SoTiSaR). METHODS: Data of 51 patients with thoracic RMS enrolled in three different CWS trials (CWS-86, -91, -2002P) and one registry (SoTiSaR) were analyzed retrospectively. Surgery and its influence on outcome were assessed. Median follow-up was 37.5 months (0.9-152.5). RESULTS: Median age of the patients was 8.8 years (range 0-19 years). The 5-year overall survival rate (OS) was 57% (95%-CI 49-65) and the 5-year event-free survival rate (EFS) was 45% (38-52). Thirty-five patients had tumors located at the chest wall (EFS: 51%, 43-59), and 16 patients had intrathoracic tumors (EFS: 26%, 13-39). Seventeen patients with tumors ≤ 5 cm had a better outcome (EFS: 64%, 52-76) compared to patients with tumors larger than 5 cm (EFS: 36%, 27-45). Radiotherapy (RT) significantly improved the survival of patients with alveolar RMS compared to patients with embryonal histology (EFS: 66%, 52-80 vs. 32%, 21-43 p = 0.02). Complete tumor excision during delayed surgery was the main prognostic factor for survival (p = 0.045). CONCLUSION: Thoracic RMS is a rare tumor entity. Completeness of tumor resection significantly improved survival of the patients.
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