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Pediatric Patients with Stage IV Rhabdomyosarcoma Significantly Benefit from Long-Term Maintenance Therapy : Results of the CWS-IV 2002 and the CWS DOK IV 2004-Trials

Tramsen, Lars (författare)
Univ Hosp Schleswig Holstein, Dept Pediat, Campus Kiel, D-24105 Kiel, Germany.;Goethe Univ, Univ Hosp, Dept Children & Adolescents, D-60590 Frankfurt, Germany.
Bochennek, Konrad (författare)
Goethe Univ, Univ Hosp, Dept Children & Adolescents, D-60590 Frankfurt, Germany.
Sparber-Sauer, Monika (författare)
Olgahosp Stuttgart Canc Ctr, Hosp State Capital Stuttgart, Ctr Pediat Adolescent & Womens Med, Pediat Oncol Hematol Immunology 5, D-70174 Stuttgart, Germany.;Univ Tubingen, Fac Med, D-72016 Tubingen, Germany.
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Salzmann-Manrique, Emilia (författare)
Goethe Univ, Univ Hosp, Dept Children & Adolescents, D-60590 Frankfurt, Germany.
Scheer, Monika (författare)
Charite Univ Med Berlin, Dept Pediat Oncol & Hematol, Augustenburger Pl 1, D-13353 Berlin, Germany.
Dantonello, Tobias (författare)
Univ Bern, Bern Univ Hosp, Dept Pediat, Div Pediat Hematol & Oncol, CH-3010 Bern, Switzerland.
Borkhardt, Arndt (författare)
Heinrich Heine Univ, Med Fac, Dept Pediat Oncol Hematol & Clin Immunol, D-40225 Dusseldorf, Germany.
Dirksen, Uta (författare)
Univ Hosp Essen, West German Canc Ctr, Pediat 3, D-45147 Essen, Germany.;Natl Ctr Tumor Dis NCT Site Essen, German Canc Consortium Site Essen, D-45147 Essen, Germany.
Thorwarth, Anne (författare)
Charite Univ Med Berlin, Dept Pediat Oncol & Hematol, Augustenburger Pl 1, D-13353 Berlin, Germany.
Greiner, Jeanette (författare)
Kantonsspital Aarau AG, Childrens Hosp, Pediat Oncol & Hematol, CH-5001 Aarau, Switzerland.
Ebinger, Martin (författare)
Univ Childrens Hosp, Dept Gen Pediat & Pediat Oncol & Hematol, D-72076 Tubingen, Germany.
Weclawek-Tompol, Jadwiga (författare)
Univ Med Wroclaw, Dept Bone Marrow Transplantat, Pediat Oncol & Haematol, Wroclaw, Poland.
Ladenstein, Ruth (författare)
St Anna Childrens Hosp, Childrens Canc Res Inst CCRI, A-1090 Vienna, Austria.
Ljungman, Gustaf, 1958- (författare)
Uppsala universitet,Barnonkologisk forskning - särskilt fokus på komplikationer,Uppsala Univ, Dept Womens & Childrens Hlth, Pediat Oncol, S-75185 Uppsala, Sweden.
Hallmen, Erika (författare)
Olgahosp Stuttgart Canc Ctr, Hosp State Capital Stuttgart, Ctr Pediat Adolescent & Womens Med, Pediat Oncol Hematol Immunology 5, D-70174 Stuttgart, Germany.
Lehrnbecher, Thomas (författare)
Goethe Univ, Univ Hosp, Dept Children & Adolescents, D-60590 Frankfurt, Germany.
Koscielniak, Ewa (författare)
Olgahosp Stuttgart Canc Ctr, Hosp State Capital Stuttgart, Ctr Pediat Adolescent & Womens Med, Pediat Oncol Hematol Immunology 5, D-70174 Stuttgart, Germany.;Univ Tubingen, Fac Med, D-72016 Tubingen, Germany.
Klingebiel, Thomas (författare)
Goethe Univ, Univ Hosp, Dept Children & Adolescents, D-60590 Frankfurt, Germany.
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Univ Hosp Schleswig Holstein, Dept Pediat, Campus Kiel, D-24105 Kiel, Germany;Goethe Univ, Univ Hosp, Dept Children & Adolescents, D-60590 Frankfurt, Germany. Goethe Univ, Univ Hosp, Dept Children & Adolescents, D-60590 Frankfurt, Germany. (creator_code:org_t)
2023-03-30
2023
Engelska.
Ingår i: Cancers. - : MDPI. - 2072-6694. ; 15:7
Relaterad länk:
https://uu.diva-port... (primary) (Raw object)
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https://urn.kb.se/re...
https://doi.org/10.3...
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  • Tidskriftsartikel (refereegranskat)
Abstract Ämnesord
Stäng  
  • Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma (STS) in childhood. Whereas more than 90% of patients with localized low-risk RMS can be cured, metastatic RMS have a dismal outcome, with survival rates of less than 30%. The HD CWS-96 trial showed an improved outcome for patients receiving maintenance therapy after completing intensive chemotherapy. Consequently, the international clinical trials CWS-IV 2002 and CWS DOK IV 2004 on metastatic disease of STS of the Cooperative Weichteilsarkom Studiengruppe (CWS) were designed in addition to the CWS-2002P trial for localized RMS disease. All patients received a multimodal intensive treatment regimen. To maintain remission, three options were compared: long-term maintenance therapy (LTMT) versus allogeneic hematopoietic stem cell transplantation (alloHSCT) versus high-dose chemotherapy (HDCT). A total of 176 pediatric patients with a histologically confirmed diagnosis of metastatic RMS or RMS-like tumor were included. A total of 89 patients receiving LTML showed a significantly better outcome, with an event-free survival (EFS) of 41% and an overall survival (OS) of 53%, than alloHSCT (n = 21, EFS 19%, p = 0.02, OS 24%, p = 0.002). The outcome of LTML was slightly improved compared to HDCT (n = 13, EFS 35%, OS 34%). In conclusion, our data suggest that in patients suffering from metastatic RMS, long-term maintenance therapy is a superior strategy in terms of EFS and OS compared to alloHSCT. EFS and OS of HDCT are similar in these strategies; however, the therapeutic burden of LTMT is much lower.

Ämnesord

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Cancer and Oncology (hsv//eng)

Nyckelord

soft tissue sarcoma
rhabdomyosarcoma
metastatic
children
long-term maintenance therapy
outcome

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  • Cancers (Sök värdpublikationen i LIBRIS)

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