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Survival following ...
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Dantonello, Tobias M.
(författare)
Survival following disease recurrence of primary localized alveolar rhabdomyosarcoma
- Artikel/kapitelEngelska2013
Förlag, utgivningsår, omfång ...
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2013-02-15
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Wiley,2013
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printrdacarrier
Nummerbeteckningar
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LIBRIS-ID:oai:DiVA.org:uu-204847
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https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-204847URI
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https://doi.org/10.1002/pbc.24488DOI
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Språk:engelska
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Sammanfattning på:engelska
Ingår i deldatabas
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Ämneskategori:ref swepub-contenttype
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Ämneskategori:art swepub-publicationtype
Anmärkningar
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On behalf of the Cooperative Weichteilsarkom Studiengruppe (CWS); Presented in part: At the International Society of Paediatric Oncology SIOP XXXX Congress, Berlin, Germany, October 2–6, 2008.
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Background Recurrences in primary localized alveolar rhabdomyosarcoma (RMA) are common. Post-relapse survival is poor. We evaluated prognostic factors including relapse treatment in patients with recurrent RMA. Methods Relapses occurred in 115/235 patients with nonmetastatic RMA treated in four consecutive CWS-trials after achievement of a complete remission. Sufficient information about post-relapse treatment and outcome could be obtained in 99 patients and was retrospectively analyzed. Results Nine of 99 patients received no salvage therapy and died after a median of 2 months. The remaining 90 patients received multimodal relapse treatment including mandatory chemotherapy. Recurrences were grossly resected in 39 patients; 57 patients received radiation. At a median follow-up from relapse of 8 years, 20 patients were alive and disease-free (5-year post-relapse survival [PROS] 21.3 +/- 8). All surviving patients apart from a single individual had an isolated, circumscribed recurrence. Sixteen of 20 survivors were treated with adequate local relapse therapy (ALRT, i.e., either complete resection or gross resection+radiation). Survival in the subgroup of 27 individuals with circumscribed recurrences and ALRT was significantly better (PROS 53.7 +/- 19) compared with disseminated recurrences and/or tumors treated without ALRT. Absence of primary lymph node involvement, circumscribed relapses, ALRT, and achievement of a second CR were identified as independent favorable risk factors. Conclusion Post-relapse survival for primary localized RMA is generally poor. However, certain patient groups differed significantly in their likelihood of survival and 50% of patients with circumscribed relapses treated with ALRT survived. These findings may form the basis for an evidence-based risk-stratification for recurrent disease including relapse treatment.
Ämnesord och genrebeteckningar
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alveolar rhabdomyosarcoma
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children
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recurrence
Biuppslag (personer, institutioner, konferenser, titlar ...)
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Int-Veen, Christoph
(författare)
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Schuck, Andreas
(författare)
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Seitz, Guido
(författare)
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Leuschner, Ivo
(författare)
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Nathrath, Michaela
(författare)
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Schlegel, Paul-Gerhardt
(författare)
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Kontny, Udo
(författare)
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Behnisch, Wolfgang
(författare)
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Veit-Friedrich, Iris
(författare)
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Kube, Stefanie
(författare)
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Hallmen, Erika
(författare)
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Kazanowska, Bernarda
(författare)
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Ladenstein, Ruth
(författare)
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Paulussen, Michael
(författare)
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Ljungman, GustafUppsala universitet,Pediatrik,Barnonkologisk forskning/Ljungman(Swepub:uu)gustaflm
(författare)
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Bielack, Stefan S.
(författare)
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Klingebiel, T.
(författare)
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Koscielniak, E.
(författare)
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Uppsala universitetPediatrik
(creator_code:org_t)
Sammanhörande titlar
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Ingår i:Pediatric Blood & Cancer: Wiley60:8, s. 1267-12731545-50091545-5017
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Dantonello, Tobi ...
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Int-Veen, Christ ...
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Schuck, Andreas
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Seitz, Guido
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Leuschner, Ivo
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Nathrath, Michae ...
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visa fler...
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Schlegel, Paul-G ...
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Kontny, Udo
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Behnisch, Wolfga ...
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Veit-Friedrich, ...
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Kube, Stefanie
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Hallmen, Erika
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Kazanowska, Bern ...
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Ladenstein, Ruth
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Paulussen, Micha ...
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Ljungman, Gustaf
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Bielack, Stefan ...
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Klingebiel, T.
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Koscielniak, E.
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Uppsala universitet