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

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
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.
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Metadaten
Author:Monika Scheer, Christian Vokuhl, Bernd Blank, Erika Hallmen, Thekla von Kalle, Marc Münter, Rüdiger Wessalowski, Maite Hartwig, Monika Sparber‐Sauer, Paul‐Gerhardt Schlegel, Christof M. Kramm, Udo Kontny, Bernd Spriewald, Thomas Kegel, Sebastian Bauer, Bernarda Kazanowska, Felix Niggli, Ruth Ladenstein, Gustaf Ljungman, Kirsi Jahnukainen, Jörg Fuchs, Stefan S. Bielack, Thomas Klingebiel, Ewa Koscielniak
URN:urn:nbn:de:hebis:30:3-488621
DOI:http://dx.doi.org/10.1002/cam4.1940
ISSN:2045-7634
Pubmed Id:http://www.ncbi.nlm.nih.gov/pubmed?term=30652419
Parent Title (English):Cancer medicine
Publisher:Wiley
Place of publication:Hoboken, NJ
Document Type:Article
Language:English
Year of Completion:2019
Date of first Publication:2019/01/16
Publishing Institution:Universitätsbibliothek Johann Christian Senckenberg
Contributing Corporation:Cooperative Weichteilsarkom Studiengruppe [CWS]
Release Date:2019/02/07
Tag:C‐reactive protein; Trousseau’s syndrome; desmoplastic small round cell tumor; maintenance therapy; soft tissue sarcoma
Volume:2019
Issue:00
Pagenumber:16
First Page:1
Last Page:16
Note:
This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
HeBIS PPN:446477656
Institutes:Medizin
Dewey Decimal Classification:610 Medizin und Gesundheit
Sammlungen:Universitätspublikationen
Licence (German):License LogoCreative Commons - Namensnennung 4.0

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