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Malignant periphera...
Malignant peripheral nerve sheath tumors in children, adolescents, and young adults : Treatment results of five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry
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- Meister, Michael T. (författare)
- Univ Hosp Frankfurt, Dept Pediat Oncol & Hematol, Frankfurt, Germany.;German Canc Consortium DKTK, Heidelberg, Germany.;German Canc Res Ctr, Heidelberg, Germany.;Princess Maxima Ctr Pediat Oncol, Res, Heidelberglaan 25, NL-3584 CS Utrecht, Netherlands.
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- Scheer, Monika (författare)
- Olga Hosp, Ctr Pediat Adolescent & Womens Med, Stuttgart Canc Ctr, Klinikum Stuttgart,Pediat Oncol Hematol Immunol 5, Stuttgart, Germany.
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- Hallmen, Erika (författare)
- Olga Hosp, Ctr Pediat Adolescent & Womens Med, Stuttgart Canc Ctr, Klinikum Stuttgart,Pediat Oncol Hematol Immunol 5, Stuttgart, Germany.
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- Stegmaier, Sabine (författare)
- Olga Hosp, Ctr Pediat Adolescent & Womens Med, Stuttgart Canc Ctr, Klinikum Stuttgart,Pediat Oncol Hematol Immunol 5, Stuttgart, Germany.
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- Vokuhl, Christian (författare)
- Univ Hosp Bonn, Dept Pathol, Sect Pediat Pathol, Bonn, Germany.
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- von Kalle, Thekla (författare)
- Olga Hosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed, Inst Radiol,Klinikum Stuttgart, Stuttgart, Germany.
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- Fuchs, Joerg (författare)
- Univ Childrens Hosp, Dept Pediat Surg & Urol, Tubingen, Germany.
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- Muenter, Marc (författare)
- Klinikum Stuttgart, Inst Radiotherapy, Stuttgart, Germany.
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- Niggli, Felix (författare)
- Univ Zurich, Dept Pediat Oncol, Zurich, Switzerland.
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- Ladenstein, Ruth (författare)
- St Anna Childrens Hosp, Dept Pediat, Vienna, Austria.;St Anna Kinderkrebsforsch eV, Vienna, Austria.
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- Kazanowska, Bernarda (författare)
- Univ Wroclaw, Dept Pediat Oncol, Wroclaw, Poland.
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- Ljungman, Gustaf, 1958- (författare)
- Uppsala universitet,Institutionen för kvinnors och barns hälsa
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- Bielack, Stefan (författare)
- Olga Hosp, Ctr Pediat Adolescent & Womens Med, Stuttgart Canc Ctr, Klinikum Stuttgart,Pediat Oncol Hematol Immunol 5, Stuttgart, Germany.
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- Koscielniak, Ewa (författare)
- Olga Hosp, Ctr Pediat Adolescent & Womens Med, Stuttgart Canc Ctr, Klinikum Stuttgart,Pediat Oncol Hematol Immunol 5, Stuttgart, Germany.
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- Klingebiel, Thomas (författare)
- Univ Hosp Frankfurt, Dept Pediat Oncol & Hematol, Frankfurt, Germany.
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Univ Hosp Frankfurt, Dept Pediat Oncol & Hematol, Frankfurt, Germany;German Canc Consortium DKTK, Heidelberg, Germany.;German Canc Res Ctr, Heidelberg, Germany.;Princess Maxima Ctr Pediat Oncol, Res, Heidelberglaan 25, NL-3584 CS Utrecht, Netherlands. Olga Hosp, Ctr Pediat Adolescent & Womens Med, Stuttgart Canc Ctr, Klinikum Stuttgart,Pediat Oncol Hematol Immunol 5, Stuttgart, Germany. (creator_code:org_t)
- 2020-08-18
- 2020
- Engelska.
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Ingår i: Journal of Surgical Oncology. - : WILEY. - 0022-4790 .- 1096-9098. ; 122:7, s. 1337-1347
- Relaterad länk:
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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 and Objectives Malignant peripheral nerve sheath tumors (MPNST) are aggressive soft tissue sarcomas that present as large, invasive tumors. Our aim was to assess outcomes, identify prognostic factors, and analyze treatment strategies in a prospectively collected pediatric cohort. Methods Patients less than 21 years with MPNST treated in the consecutive prospective European Cooperative Weichteilsarkom Studiengruppe (CWS)-trials (1981-2009) and the CWS-SoTiSaR registry (2009-2015) were analyzed. Results A total of 159 patients were analyzed. Neurofibromatosis type I (NF1) was reported in thirty-eight patients (24%). Most were adolescents (67%) with large (>10 cm, 65%) tumors located at extremities (42%). Nodal involvement was documented in 15 (9%) and distant metastases in 15 (9%) upon diagnosis. Overall, event-free survival (EFS) was 40.5% at 5 and 36.3% at 10 years, and overall survival (OS) was 54.6% at 5 and 47.1% at 10 years. Age, NF1 status, tumor site, tumor size, Intergroup Rhabdomyosarcoma Study (IRS) group, metastatic disease, and achieving first complete remission (CR1) were identified as prognostic factors for EFS and/or OS in the univariate analysis. Conclusions Prognostic factors were identified and research questions for future clinical trials were addressed.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cancer and Oncology (hsv//eng)
Nyckelord
- malignant peripheral nerve sheath tumor
- metastatic sarcoma
- neurofibromatosis
- pediatric sarcoma
- soft tissue sarcoma
Publikations- och innehållstyp
- ref (ämneskategori)
- art (ämneskategori)
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Till lärosätets databas
- Av författaren/redakt...
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Meister, Michael ...
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Scheer, Monika
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Hallmen, Erika
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Stegmaier, Sabin ...
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Vokuhl, Christia ...
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von Kalle, Thekl ...
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visa fler...
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Fuchs, Joerg
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Muenter, Marc
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Niggli, Felix
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Ladenstein, Ruth
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Kazanowska, Bern ...
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Ljungman, Gustaf ...
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Bielack, Stefan
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Koscielniak, Ewa
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Klingebiel, Thom ...
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MEDICIN OCH HÄLS ...
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Journal of Surgi ...
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Uppsala universitet