Challenges in the Local Treatment of Large Abdominal Embryonal Rhabdomyosarcoma |
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Authors: | Tobias M. Dantonello MD Helmut Lochbühler MD Andreas Schuck MD Stefanie Kube MD Jan Godzinski MD Erik Sköldenberg MD Gustaf Ljungman MD Daniel Kosztyla Dipl-Inform Iris Veit-Friedrich CDM Erika Hallmen CDM Simone Feuchtgruber CDM Ruediger Wessalowski MD Markus Franke MD Stefan S. Bielack MD Thomas Klingebiel MD Ewa Koscielniak MD |
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Affiliation: | 1. Pediatrics 5 (Oncology, Hematology, Immunology, Rheumatology, Gastroenterology and General Pedatrics), Olgahospital, Klinikum Stuttgart, Stuttgart, Germany 2. Department of Pediatric Surgery, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany 3. Department of Radiotherapy, University of Muenster, Münster, Germany 4. Department of Pediatric Surgery, University of Wroclaw, Wroclaw, Poland 5. Department of Women’s and Children’s Health, University of Uppsala, Uppsala, Sweden 6. Department of Pediatric Oncology, University of Duesseldorf, Düsseldorf, Germany 7. Department of Pediatric Surgery, University of Freiburg, Freiburg, Germany 8. Department of Pediatric Hematology and Oncology, University Children’s Hospital Muenster, Münster, Germany 9. Department of Pediatric Oncology, University of Frankfurt, Frankfurt (Main), Germany 10. Department of Pediatric Oncology, University of Tuebingen, Tübingen, Germany
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Abstract: | Background Embryonal rhabdomyosarcoma is the most common pediatric soft tissue sarcoma. The best local treatment in large, nonmetastatic primary unresected nongenitourinary embryonal rhabdomyosarcoma of the abdomen (LARME) is however unclear. Methods We analyzed patients with LARME treated in four consecutive CWS trials. All diagnoses were confirmed by reference reviews. Treatment included multiagent chemotherapy and local treatment of the primary tumor with surgery and/or radiotherapy. The impact of primary debulking surgery (PDS) also was studied. Results One hundred patients <21 years with a median age of 4 years had LARME. Sixty-one of them had a tumor >10 cm in diameter at diagnosis. PDS was performed in 19 of 100 children. The outcomes of patients with PDS were similar to those of the other patients. In 36 children, the tumor was resected after induction chemotherapy; 60 RME were irradiated. The toxic effects of radiochemotherapy were not significantly increased compared with the nonirradiated patients. With a median follow-up of 10 years, the 5-year EFS and OS were 52 ± 10 and 65 ± 9 %, respectively. Significant risk factors in multivariate analysis were age >10 years; no achievement of complete remission; and inadequate secondary local treatment, defined as incomplete secondary resection or no radiation. Conclusions Children with LARME have a fair prognosis, despite an often huge tumor size and unfavorable primary site, if the tumors can either be resected or irradiated following induction chemotherapy. PDS was only performed in a small subgroup. Radiation performed concomitantly with chemotherapy did not increase the acute toxicity significantly. |
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