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Desmoplastic small ...
Desmoplastic small round cell tumors : Multimodality treatment and new risk factors
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- Scheer, Monika (author)
- Klinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany
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- Vokuhl, Christian (author)
- Univ Hosp Kiel, Dept Pediat Pathol, Kiel Peadiat Tumour Registry, Kiel, Germany
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- Blank, Bernd (author)
- Klinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany
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- Hallmen, Erika (author)
- Klinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany
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- von Kalle, Thekla (author)
- Klinikum Stuttgart, Radiol Inst, Olgahosp, Stuttgart, Germany
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- Muenter, Marc (author)
- Klinikum Stuttgart, Radiat Oncol, Stuttgart, Germany
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- Wessalowski, Ruediger (author)
- Heinrich Heine Univ Dusseldorf, Pediat Oncol Clin, Dusseldorf, Germany
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- Hartwig, Maite (author)
- Univ Hamburg Eppendorf, Pediat Hematol & Oncol, Hamburg, Germany
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- Sparber-Sauer, Monika (author)
- Klinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany
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- Schlegel, Paul-Gerhardt (author)
- Univ Wurzburg, Childrens Hosp, Pediat Oncol, Wurzburg, Germany
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- Kramm, Christof M. (author)
- Univ Med Ctr Gottingen, Pediat Hematol & Oncol, Gottingen, Germany
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- Kontny, Udo (author)
- Univ Med Ctr Aachen, Pediat Hematol & Oncol, Aachen, Germany
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- Spriewald, Bernd (author)
- Univ Hosp Erlangen, Internal Med 5, Erlangen, Germany
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- Kegel, Thomas (author)
- Univ Halle, Hematol Oncol, Halle, Germany
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- Bauer, Sebastian (author)
- Univ Duisburg Essen, West German Canc Ctr, Sarcoma Ctr, Essen, Germany
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- Kazanowska, Bernarda (author)
- Univ Wroclaw, Dept Pediat Hematol Oncol, Wroclaw, Poland; Univ Wroclaw, BMT, Wroclaw, Poland
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- Niggli, Felix (author)
- Univ Zurich, Pediat Oncol, Zurich, Switzerland
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- Ladenstein, Ruth (author)
- St Anna Childrens Hosp, Vienna, Austria; St Anna Kinderkrebsforsch eV, Vienna, Austria
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- Ljungman, Gustaf, 1958- (author)
- Uppsala universitet,Barnneurologi/Barnonkologi
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- Jahnukainen, Kirsi (author)
- Univ Helsinki, Pediat, Cent Hosp, Helsinki, Finland
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- Fuchs, Joerg (author)
- Univ Hosp Tubingen, Pediat Surg, Tubingen, Germany
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- Bielack, Stefan S. (author)
- Klinikum Stuttgart, Olgahosp, Pediat 5, Stuttgart, Germany; Univ Childrens Hosp Munster, Pediat Hematol & Oncol, Munster, Germany
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- Klingebiel, Thomas (author)
- Goethe Univ Frankfurt, Univ Hosp, Dept Children & Adolescents, Frankfurt, Germany
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- Koscielniak, Ewa (author)
- Univ Tubingen, Pediat Hematol & Oncol, Tubingen, Germany
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(creator_code:org_t)
- 2019-01-16
- 2019
- English.
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In: Cancer Medicine. - : WILEY. - 2045-7634. ; 8:2, s. 527-542
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Abstract
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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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cancer and Oncology (hsv//eng)
Keyword
- C-reactive protein
- desmoplastic small round cell tumor
- maintenance therapy
- soft tissue sarcoma
- Trousseau's syndrome
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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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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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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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Cancer and Oncol ...
- Articles in the publication
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Cancer Medicine
- By the university
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Uppsala University