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Embryonal rhabdomyo...
Embryonal rhabdomyosarcoma with metastases confined to the lungs : Report from the CWS Study Group.
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- Dantonello, Tobias M (author)
- Pediatrics 5 (Oncology, Hematology, Immunology), Olgahospital, Klinikum Stuttgart, Germany
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- Winkler, Peter (author)
- Department of Pediatric Radiology, Olgahospital, Klinikum Stuttgart, Germany
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- Boelling, Tobias (author)
- Department of Radiotherapy, University of Muenster, Muenster, Germany
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- Friedel, Godehard (author)
- Department of Thoracic Surgery, Klinik Schillerhoehe, Gerlingen, Germany
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- Schmid, Irene (author)
- Department of Pediatric Oncology, Dr. von Haunersches Kinderspital, University of Munich, Munich, Germany
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- Mattke, Adrian C (author)
- Department for Pediatric Intensive Care Medicine, Royal Children’s Hospital, Parkville, Victoria, Australia
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- Ljungman, Gustaf (author)
- Uppsala universitet,Pediatrik,Barnonkologisk forskning/Pfeifer
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- Bielack, Stefan S (author)
- Pediatrics 5 (Oncology, Hematology, Immunology), Olgahospital, Klinikum Stuttgart, Germany
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- Klingebiel, Thomas (author)
- Department of Pediatric Oncology, University of Frankfurt, Frankfurt (Main), Germany
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- Koscielniak, Ewa (author)
- Pediatrics 5 (Oncology, Hematology, Immunology), Olgahospital, Klinikum Stuttgart, Germany
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(creator_code:org_t)
- 2010-11-08
- 2011
- English.
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In: Pediatric Blood & Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 56:5, s. 725-732
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
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- BACKGROUND: Embryonal rhabdomyosarcoma [RME] is the most common pediatric soft tissue sarcoma. Whereas the prognosis of localized rhabdomyosarcoma has improved, it remains poor for metastatic disease. METHODS: We analyzed RME-patients with isolated pulmonary metastases [PRME] treated in four consecutive CWS-trials. Treatment included multiagent chemotherapy and local treatment of the primary tumor. Therapy of lung metastases after induction chemotherapy depended on response and individual decisions. RESULTS: Twenty-nine patients <21 years had PRME. Their median age was six years, the median follow-up nine years. Twenty-eight children had their primary tumor located in an unfavorable site and 22 of the primaries were >5 cm. In addition to conventional chemotherapy, seven patients received high-dose treatment and eight patients oral metronomic chemotherapy. The lung metastases were in remission after induction chemotherapy in 22 individuals. 19 patients received no local treatment of metastases; 3 patients had pulmonary metastasectomy and lung radiation was administered to 9 individuals. In total, 24/29 patients achieved a complete remission [CR]. Actuarial 5-year event-free and overall survival for all patients was 37.9 ± 18% and 48.7 ± 18%, respectively; it was 45.8 ± 20% and 58.3 ± 20% for the 24 patients who achieved a CR. Local treatment of metastases had no impact on the failure pattern. Younger age, good response, achievement of CR and maintenance-treatment were favorable prognostic factors in univariate analysis. CONCLUSIONS: Children with PRME have a fair prognosis. Local treatment of metastases did not improve outcome in our sample. Metronomic treatment may be an attractive option for PREM-patients. [correction made here after initial online publication].
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Dantonello, Tobi ...
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Winkler, Peter
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Boelling, Tobias
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Friedel, Godehar ...
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Schmid, Irene
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Mattke, Adrian C
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show more...
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Ljungman, Gustaf
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Bielack, Stefan ...
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Klingebiel, Thom ...
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Koscielniak, Ewa
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Pediatric Blood ...
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Uppsala University