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

Journal article

Publication Details

Author(s): Scheer M, Vokuhl C, Blank B, Hallmen E, Von Kalle T, Muenter M, Wessalowski R, Hartwig M, Sparber-Sauer M, Schlegel PG, Kramm CM, Kontny U, Spriewald B, Kegel T, Bauer S, Kazanowska B, Niggli F, Ladenstein R, Ljungman G, Jahnukainen K, Fuchs J, Bielack SS, Klingebiel T, Koscielniak E
Journal: Cancer Medicine
Publication year: 2019
Volume: 8
Journal issue: 2
Pages range: 527-542
ISSN: 2045-7634


Background To evaluate optimal therapy and potential risk factors. Methods Data of DSRCT patients 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.

External institutions with authors

Eberhard Karls Universität Tübingen
Goethe-Universität Frankfurt am Main
Heinrich-Heine-Universität Düsseldorf
Helsingin yliopisto / University of Helsinki
Julius-Maximilians-Universität Würzburg
Klinikum Stuttgart
Martin-Luther-Universität Halle-Wittenberg (MLU)
St. Anna Kinderkrebsforschung
Universität Duisburg-Essen (UDE)
Universitätsklinikum Aachen
Universitätsklinikum Göttingen
Universitätsklinikum Hamburg-Eppendorf (UKE)
Universitätsklinikum Schleswig-Holstein (UKSH)
Universitätsklinikum Tübingen
Universität Zürich (UZH)
University of Wroclaw
Uppsala University

How to cite

Scheer, M., Vokuhl, C., Blank, B., Hallmen, E., Von Kalle, T., Muenter, M.,... Koscielniak, E. (2019). Desmoplastic small round cell tumors: Multimodality treatment and new risk factors. Cancer Medicine, 8(2), 527-542.

Scheer, Monika, et al. "Desmoplastic small round cell tumors: Multimodality treatment and new risk factors." Cancer Medicine 8.2 (2019): 527-542.


Last updated on 2019-13-03 at 11:38