@article{ScheerVokuhlBlanketal.2019,
  author    = {Scheer, Monika and Vokuhl, Christian and Blank, Bernd and Hallmen, Erika and von Kalle, Thekla and M{\"u}nter, Marc and Wessalowski, R{\"u}diger and Hartwig, Maite and Sparber-Sauer, Monika and Schlegel, Paul-Gerhardt and Kramm, Christof M. and Kontny, Udo and Spriewald, Bernd and Kegel, Thomas and Bauer, Sebastian and Kazanowska, Bernarda and Niggli, Felix and Ladenstein, Ruth and Ljungman, Gustaf and Jahnukainen, Kirsi and Fuchs, J{\"o}rg and Bielack, Stefan S. and Klingebiel, Thomas and Koscielniak, Ewa},
  title     = {Desmoplastic small round cell tumors: Multimodality treatment and new risk factors},
  series = {Cancer Medicine},
  volume    = {8},
  journal   = {Cancer Medicine},
  number    = {2},
  doi       = {10.1002/cam4.1940},
  url       = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-228444},
  pages     = {527-545},
  year      = {2019},
  abstract  = {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.},
  language  = {en}
}