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Desmoplastic small ...
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Scheer, MonikaKlinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany
(författare)
Desmoplastic small round cell tumors : Multimodality treatment and new risk factors
- Artikel/kapitelEngelska2019
Förlag, utgivningsår, omfång ...
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2019-01-16
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WILEY,2019
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electronicrdacarrier
Nummerbeteckningar
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LIBRIS-ID:oai:DiVA.org:uu-378728
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https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-378728URI
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https://doi.org/10.1002/cam4.1940DOI
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Språk:engelska
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Sammanfattning på:engelska
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Ämneskategori:ref swepub-contenttype
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Ämneskategori:art swepub-publicationtype
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Background: To evaluate optimal therapy and potential risk factors.Methods: Data of DSRCT patients <40 years treated in prospective CWS trials 1997‐2015 were analyzed.Results: Median age of 60 patients was 14.5 years. Male:female ratio was 4:1. Tumors were abdominal/retroperitoneal in 56/60 (93%). 6/60 (10%) presented with a localized mass, 16/60 (27%) regionally disseminated nodes, and 38/60 (63%) with extraperitoneal metastases. At diagnosis, 23/60 (38%) patients had effusions, 4/60 (7%) a thrombosis, and 37/54 (69%) elevated CRP. 40/60 (67%) patients underwent tumor resection, 21/60 (35%) macroscopically complete. 37/60 (62%) received chemotherapy according to CEVAIE (ifosfamide, vincristine, actinomycin D, carboplatin, epirubicin, etoposide), 15/60 (25%) VAIA (ifosfamide, vincristine, adriamycin, actinomycin D) and, 5/60 (8%) P6 (cyclophosphamide, doxorubicin, vincristine, ifosfamide, etoposide). Nine received high‐dose chemotherapy, 6 received regional hyperthermia, and 20 received radiotherapy. Among 25 patients achieving complete remission, 18 (72%) received metronomic therapies. Three‐year event‐free (EFS) and overall survival (OS) were 11% (±8 confidence interval [CI] 95%) and 30% (±12 CI 95%), respectively, for all patients and 26.7% (±18.0 CI 95%) and 56.9% (±20.4 CI 95%) for 25 patients achieving remission. Extra‐abdominal site, localized disease, no effusion or ascites only, absence of thrombosis, normal CRP, complete tumor resection, and chemotherapy with VAIA correlated with EFS in univariate analysis. In multivariate analysis, significant factors were no thrombosis and chemotherapy with VAIA. In patients achieving complete remission, metronomic therapy with cyclophosphamide/vinblastine correlated with prolonged time to relapse.Conclusion: Pleural effusions, venous thrombosis, and CRP elevation were identified as potential risk factors. The VAIA scheme showed best outcome. Maintenance therapy should be investigated further.
Ämnesord och genrebeteckningar
Biuppslag (personer, institutioner, konferenser, titlar ...)
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Vokuhl, ChristianUniv Hosp Kiel, Dept Pediat Pathol, Kiel Peadiat Tumour Registry, Kiel, Germany
(författare)
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Blank, BerndKlinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany
(författare)
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Hallmen, ErikaKlinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany
(författare)
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von Kalle, TheklaKlinikum Stuttgart, Radiol Inst, Olgahosp, Stuttgart, Germany
(författare)
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Muenter, MarcKlinikum Stuttgart, Radiat Oncol, Stuttgart, Germany
(författare)
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Wessalowski, RuedigerHeinrich Heine Univ Dusseldorf, Pediat Oncol Clin, Dusseldorf, Germany
(författare)
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Hartwig, MaiteUniv Hamburg Eppendorf, Pediat Hematol & Oncol, Hamburg, Germany
(författare)
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Sparber-Sauer, MonikaKlinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany
(författare)
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Schlegel, Paul-GerhardtUniv Wurzburg, Childrens Hosp, Pediat Oncol, Wurzburg, Germany
(författare)
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Kramm, Christof M.Univ Med Ctr Gottingen, Pediat Hematol & Oncol, Gottingen, Germany
(författare)
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Kontny, UdoUniv Med Ctr Aachen, Pediat Hematol & Oncol, Aachen, Germany
(författare)
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Spriewald, BerndUniv Hosp Erlangen, Internal Med 5, Erlangen, Germany
(författare)
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Kegel, ThomasUniv Halle, Hematol Oncol, Halle, Germany
(författare)
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Bauer, SebastianUniv Duisburg Essen, West German Canc Ctr, Sarcoma Ctr, Essen, Germany
(författare)
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Kazanowska, BernardaUniv Wroclaw, Dept Pediat Hematol Oncol, Wroclaw, Poland; Univ Wroclaw, BMT, Wroclaw, Poland
(författare)
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Niggli, FelixUniv Zurich, Pediat Oncol, Zurich, Switzerland
(författare)
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Ladenstein, RuthSt Anna Childrens Hosp, Vienna, Austria; St Anna Kinderkrebsforsch eV, Vienna, Austria
(författare)
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Ljungman, Gustaf,1958-Uppsala universitet,Barnneurologi/Barnonkologi(Swepub:uu)gustaflm
(författare)
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Jahnukainen, KirsiUniv Helsinki, Pediat, Cent Hosp, Helsinki, Finland
(författare)
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Fuchs, JoergUniv Hosp Tubingen, Pediat Surg, Tubingen, Germany
(författare)
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Bielack, Stefan S.Klinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany; Univ Childrens Hosp Munster, Pediat Hematol & Oncol, Munster, Germany
(författare)
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Klingebiel, ThomasGoethe Univ Frankfurt, Univ Hosp, Dept Children & Adolescents, Frankfurt, Germany
(författare)
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Koscielniak, EwaUniv Tubingen, Pediat Hematol & Oncol, Tubingen, Germany
(författare)
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Klinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, GermanyUniv Hosp Kiel, Dept Pediat Pathol, Kiel Peadiat Tumour Registry, Kiel, Germany
(creator_code:org_t)
Sammanhörande titlar
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Ingår i:Cancer Medicine: WILEY8:2, s. 527-5422045-7634
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Till lärosätets databas
- Av författaren/redakt...
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Scheer, Monika
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Vokuhl, Christia ...
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Blank, Bernd
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Hallmen, Erika
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von Kalle, Thekl ...
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Muenter, Marc
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visa fler...
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Wessalowski, Rue ...
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Hartwig, Maite
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Sparber-Sauer, M ...
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Schlegel, Paul-G ...
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Kramm, Christof ...
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Kontny, Udo
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Spriewald, Bernd
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Kegel, Thomas
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Bauer, Sebastian
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Kazanowska, Bern ...
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Niggli, Felix
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Ladenstein, Ruth
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Ljungman, Gustaf ...
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Jahnukainen, Kir ...
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Fuchs, Joerg
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Bielack, Stefan ...
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Klingebiel, Thom ...
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Koscielniak, Ewa
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