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Challenges in the L...
Challenges in the Local Treatment of Large Abdominal Embryonal Rhabdomyosarcoma
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- Dantonello, Tobias M (author)
- Pediatrics 5, Olgahospital, Klinikum Stuttgart, Germany
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- Lochbühler, Helmut (author)
- Department of Pediatric Surgery, Olgahospital, Klinikum Stuttgart, Germany
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- Schuck, Andreas (author)
- Department of Radiotherapy, University of Muenster, Münster, Germany
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- Kube, Stefanie (author)
- Pediatrics 5, Olgahospital, Klinikum Stuttgart, Germany
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- Godzinski, Jan (author)
- Department of Pediatric Surgery, University of Wroclaw, Poland
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- Sköldenberg, Erik, 1967- (author)
- Uppsala universitet,Barnkirurgi,Christofferson
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- Ljungman, Gustaf, 1958- (author)
- Uppsala universitet,Pediatrik,Barnonkologisk forskning/Ljungman
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- Kosztyla, Daniel (author)
- Pediatrics 5, Olgahospital, Klinikum Stuttgart, Germany
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- Veit-Friedrich, Iris (author)
- Pediatrics 5, Olgahospital, Klinikum Stuttgart, Germany
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- Hallmen, Erika (author)
- Pediatrics 5, Olgahospital, Klinikum Stuttgart, Germany
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- Feuchtgruber, Simone (author)
- Pediatrics 5, Olgahospital, Klinikum Stuttgart, Germany
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- Wessalowski, Ruediger (author)
- Department of Pediatric Oncology, University of Duesseldorf, Germany
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- Franke, Markus (author)
- Department of Pediatric Surgery, University of Freiburg, Germany
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- Bielack, Stefan S (author)
- Pediatrics 5, Olgahospital, Klinikum Stuttgart, Germany
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- Klingebiel, Thomas (author)
- Department of Pediatric Oncology, University of Frankfurt, Germany
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- Koscielniak, Ewa (author)
- Pediatrics 5, Olgahospital, Klinikum Stuttgart, Germany
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(creator_code:org_t)
- 2014-07-22
- 2014
- English.
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In: Annals of Surgical Oncology. - : Springer Science and Business Media LLC. - 1068-9265 .- 1534-4681. ; 21:11, s. 3579-3586
- Related links:
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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 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cancer and Oncology (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Kirurgi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Surgery (hsv//eng)
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- art (subject category)
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Dantonello, Tobi ...
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Lochbühler, Helm ...
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Schuck, Andreas
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Kube, Stefanie
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Godzinski, Jan
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Sköldenberg, Eri ...
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Ljungman, Gustaf ...
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Kosztyla, Daniel
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Veit-Friedrich, ...
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Hallmen, Erika
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Feuchtgruber, Si ...
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Wessalowski, Rue ...
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Franke, Markus
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Bielack, Stefan ...
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Klingebiel, Thom ...
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Koscielniak, Ewa
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Surgery
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Uppsala University