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Sökning: onr:"swepub:oai:DiVA.org:uu-378728" > Desmoplastic small ...

Desmoplastic small round cell tumors : Multimodality treatment and new risk factors

Scheer, Monika (författare)
Klinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany
Vokuhl, Christian (författare)
Univ Hosp Kiel, Dept Pediat Pathol, Kiel Peadiat Tumour Registry, Kiel, Germany
Blank, Bernd (författare)
Klinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany
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Hallmen, Erika (författare)
Klinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany
von Kalle, Thekla (författare)
Klinikum Stuttgart, Radiol Inst, Olgahosp, Stuttgart, Germany
Muenter, Marc (författare)
Klinikum Stuttgart, Radiat Oncol, Stuttgart, Germany
Wessalowski, Ruediger (författare)
Heinrich Heine Univ Dusseldorf, Pediat Oncol Clin, Dusseldorf, Germany
Hartwig, Maite (författare)
Univ Hamburg Eppendorf, Pediat Hematol & Oncol, Hamburg, Germany
Sparber-Sauer, Monika (författare)
Klinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany
Schlegel, Paul-Gerhardt (författare)
Univ Wurzburg, Childrens Hosp, Pediat Oncol, Wurzburg, Germany
Kramm, Christof M. (författare)
Univ Med Ctr Gottingen, Pediat Hematol & Oncol, Gottingen, Germany
Kontny, Udo (författare)
Univ Med Ctr Aachen, Pediat Hematol & Oncol, Aachen, Germany
Spriewald, Bernd (författare)
Univ Hosp Erlangen, Internal Med 5, Erlangen, Germany
Kegel, Thomas (författare)
Univ Halle, Hematol Oncol, Halle, Germany
Bauer, Sebastian (författare)
Univ Duisburg Essen, West German Canc Ctr, Sarcoma Ctr, Essen, Germany
Kazanowska, Bernarda (författare)
Univ Wroclaw, Dept Pediat Hematol Oncol, Wroclaw, Poland; Univ Wroclaw, BMT, Wroclaw, Poland
Niggli, Felix (författare)
Univ Zurich, Pediat Oncol, Zurich, Switzerland
Ladenstein, Ruth (författare)
St Anna Childrens Hosp, Vienna, Austria; St Anna Kinderkrebsforsch eV, Vienna, Austria
Ljungman, Gustaf, 1958- (författare)
Uppsala universitet,Barnneurologi/Barnonkologi
Jahnukainen, Kirsi (författare)
Univ Helsinki, Pediat, Cent Hosp, Helsinki, Finland
Fuchs, Joerg (författare)
Univ Hosp Tubingen, Pediat Surg, Tubingen, Germany
Bielack, Stefan S. (författare)
Klinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany; Univ Childrens Hosp Munster, Pediat Hematol & Oncol, Munster, Germany
Klingebiel, Thomas (författare)
Goethe Univ Frankfurt, Univ Hosp, Dept Children & Adolescents, Frankfurt, Germany
Koscielniak, Ewa (författare)
Univ Tubingen, Pediat Hematol & Oncol, Tubingen, Germany
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 (creator_code:org_t)
WILEY, 2019
2019
Engelska.
Ingår i: Cancer Medicine. - : WILEY. - 2045-7634 .- 2045-7634. ; 8:2, s. 527-542
  • Tidskriftsartikel (refereegranskat)
Abstract Ämnesord
Stäng  
  • 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

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

Nyckelord

C-reactive protein
desmoplastic small round cell tumor
maintenance therapy
soft tissue sarcoma
Trousseau's syndrome

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