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Significance of fus...
Significance of fusion status, Oberlin risk factors, local and maintenance treatment in pediatric and adolescent patients with metastatic rhabdomyosarcoma : Data of the European Soft Tissue Sarcoma Registry SoTiSaR
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- Heinz, Amadeus T. (författare)
- Univ Childrens Hosp Tuebingen, Dept Pediat Hematol & Oncol, Tubingen, Germany.;Klinikum Landeshauptstadt Stuttgart, Stuttgart Canc Ctr, Padiat Padiat Onkol Hamatol Immunol 5, Zentrum Kinder Jugend & Frauenmed Olgahosp, Stuttgart, Germany.
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- Schoenstein, Anton (författare)
- Heidelberg Univ, Network Aging Res, Heidelberg, Germany.
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- Ebinger, Martin (författare)
- Univ Childrens Hosp Tuebingen, Dept Pediat Hematol & Oncol, Tubingen, Germany.
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- Fuchs, Joerg (författare)
- Univ Childrens Hosp, Dept Pediat Surg & Urol, Tubingen, Germany.
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- Timmermann, Beate (författare)
- Univ Med Ctr Essen, German Canc Consortium DKTK, West German Canc Ctr WTZ, Dept Particle Therapy,West German Proton Therapy, Essen, Germany.
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- Seitz, Guido (författare)
- Univ Hosp Giessen Marburg, Dept Pediat Surg & Urol, Marburg, Germany.
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- Vokuhl, Christian (författare)
- Dept Pathol, Sect Pediat Pathol, Bonn, Germany.
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- Muenter, Marc (författare)
- Klinikum Landeshauptstadt Stuttgart, Dept Radiat Oncol, Stuttgart Canc Ctr, Stuttgart, Germany.
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- Pajtler, Kristian W. (författare)
- Heidelberg Univ, Hopp Childrens Canc Ctr Heidelberg KiTZ, Heidelberg, Germany.;German Canc Res Ctr, Div Pediat Neurooncol, Heidelberg, Germany.;Heidelberg Univ, German Canc Consortium DKTK, Heidelberg, Germany.;Heidelberg Univ Hosp, Dept Pediat Oncol Hematol & Immunol, Heidelberg, Germany.
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- Stegmaier, Sabine (författare)
- Klinikum Landeshauptstadt Stuttgart, Stuttgart Canc Ctr, Padiat Padiat Onkol Hamatol Immunol 5, Zentrum Kinder Jugend & Frauenmed Olgahosp, Stuttgart, Germany.
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- von Kalle, Thekla (författare)
- Klinikum Landeshauptstadt Stuttgart, Zentrum Kinder Jugend & Frauenmed Olgahosp, Dept Radiol, Stuttgart Canc Ctr, Stuttgart, Germany.
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- Kratz, Christian P. (författare)
- Hannover Med Sch, Pediat Hematol & Oncol, Hannover, Germany.
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- Ljungman, Gustaf, 1958- (författare)
- Uppsala universitet,Barnonkologisk forskning - särskilt fokus på komplikationer
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- Juntti, Hanna (författare)
- Oulu Univ Hosp, Dept Pediat & Adolescence, Oulu, Finland.
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- Klingebiel, Thomas (författare)
- Univ Hosp Frankfurt, Dept Children & Adolescents, Frankfurt, Germany.
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- Koscielniak, Ewa (författare)
- Klinikum Landeshauptstadt Stuttgart, Stuttgart Canc Ctr, Padiat Padiat Onkol Hamatol Immunol 5, Zentrum Kinder Jugend & Frauenmed Olgahosp, Stuttgart, Germany.;Univ Tubingen, Med Fac, Tubingen, Germany.
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- Sparber-Sauer, Monika (författare)
- Klinikum Landeshauptstadt Stuttgart, Stuttgart Canc Ctr, Padiat Padiat Onkol Hamatol Immunol 5, Zentrum Kinder Jugend & Frauenmed Olgahosp, Stuttgart, Germany.;Univ Tubingen, Med Fac, Tubingen, Germany.
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Univ Childrens Hosp Tuebingen, Dept Pediat Hematol & Oncol, Tubingen, Germany;Klinikum Landeshauptstadt Stuttgart, Stuttgart Canc Ctr, Padiat Padiat Onkol Hamatol Immunol 5, Zentrum Kinder Jugend & Frauenmed Olgahosp, Stuttgart, Germany. Heidelberg Univ, Network Aging Res, Heidelberg, Germany. (creator_code:org_t)
- John Wiley & Sons, 2024
- 2024
- Engelska.
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Ingår i: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 71:1
- Relaterad länk:
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https://doi.org/10.1...
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https://uu.diva-port... (primary) (Raw object)
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
Ämnesord
Stäng
- Background: Outcome of primary metastatic rhabdomyosarcoma (RMS) is poor. Certain risk factors as fusion status, Oberlin score, and local treatment of primary tumor are known to influence prognosis.Procedure: Patients with metastatic RMS were treated according to Cooperative Weichteilsarkom Studiengruppe (CWS) guidance with chemotherapy (CHT), radiotherapy (RT) excluding total lung irradiation (TLI), complete resection of the primary tumor, and metastasectomy if possible. Kaplan-Meier estimators and Cox proportional hazard models were used to examine event-free survival (EFS) and overall survival (OS) involving also landmark analyses.Results: In the European Soft Tissue Sarcoma Registry SoTiSaR (2009-2018), 211 patients were analyzed. Many patients had fusion-positive alveolar RMS (n = 83; 39%). Median age was 9.4 years [0.1-19.7 years]. Treatment primarily consisted of CHT with CEVAIE (carboplatin, epirubicine, vincristine, actinomycin-D, ifosfamide, etoposide: 86%, other regimens: 14%), RT (71%), resection of primary tumor (37%), metastasectomy (19%), and lymph node sampling/dissection (21%). Maintenance treatment (MT) (oral trofosfamide, idarubicin, etoposide) was added in 74% of patients. Oberlin factors, fusion status, and MT were predictive for EFS and OS. MT with O-TIE was not improving outcome when adjusting for the immortal time bias. Local treatment of the primary tumor and radical irradiation (except TLI) improved EFS, not OS, when adjusting for the Oberlin score. Patients with fusion-negative alveolar RMS (n = 9) had an excellent outcome with a 5-year EFS and OS of 100%, compared to patients with embryonal RMS (49%/62%), PAX7- (22%/47%) and PAX3/FOXO1-positive ARMS (10/13%), respectively (p < .001).Conclusions: Prognosis of metastatic RMS primarily depends on fusion status and Oberlin score. Fusion status needs to be considered in future trials to optimize treatment outcome. The role of radical irradiation needs further investigation.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cancer and Oncology (hsv//eng)
Nyckelord
- fusion status
- local treatment
- metastatic disease
- Oberlin score
- pediatric/adolescent oncology
- rhabdomyosarcoma
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Heinz, Amadeus T ...
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Schoenstein, Ant ...
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Ebinger, Martin
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Fuchs, Joerg
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Timmermann, Beat ...
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Seitz, Guido
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Vokuhl, Christia ...
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Muenter, Marc
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Pajtler, Kristia ...
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Stegmaier, Sabin ...
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von Kalle, Thekl ...
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Kratz, Christian ...
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Ljungman, Gustaf ...
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Juntti, Hanna
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Klingebiel, Thom ...
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Koscielniak, Ewa
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Sparber-Sauer, M ...
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