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  • Dantonello, Tobias M. (författare)

Survival following disease recurrence of primary localized alveolar rhabdomyosarcoma

  • Artikel/kapitelEngelska2013

Förlag, utgivningsår, omfång ...

  • 2013-02-15
  • Wiley,2013
  • printrdacarrier

Nummerbeteckningar

  • LIBRIS-ID:oai:DiVA.org:uu-204847
  • https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-204847URI
  • https://doi.org/10.1002/pbc.24488DOI

Kompletterande språkuppgifter

  • Språk:engelska
  • Sammanfattning på:engelska

Ingår i deldatabas

Klassifikation

  • Ämneskategori:ref swepub-contenttype
  • Ämneskategori:art swepub-publicationtype

Anmärkningar

  • 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.
  • 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

  • alveolar rhabdomyosarcoma
  • children
  • recurrence

Biuppslag (personer, institutioner, konferenser, titlar ...)

  • Int-Veen, Christoph (författare)
  • Schuck, Andreas (författare)
  • Seitz, Guido (författare)
  • Leuschner, Ivo (författare)
  • Nathrath, Michaela (författare)
  • Schlegel, Paul-Gerhardt (författare)
  • Kontny, Udo (författare)
  • Behnisch, Wolfgang (författare)
  • Veit-Friedrich, Iris (författare)
  • Kube, Stefanie (författare)
  • Hallmen, Erika (författare)
  • Kazanowska, Bernarda (författare)
  • Ladenstein, Ruth (författare)
  • Paulussen, Michael (författare)
  • Ljungman, GustafUppsala universitet,Pediatrik,Barnonkologisk forskning/Ljungman(Swepub:uu)gustaflm (författare)
  • Bielack, Stefan S. (författare)
  • Klingebiel, T. (författare)
  • Koscielniak, E. (författare)
  • Uppsala universitetPediatrik (creator_code:org_t)

Sammanhörande titlar

  • Ingår i:Pediatric Blood & Cancer: Wiley60:8, s. 1267-12731545-50091545-5017

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