Desmoplastic small round cell tumors : multimodality treatment and new risk factors
- Scheer, Monika
- Vokuhl, Christian
- Blank, Bernd
- Hallmen, Erika
- Kalle, Thekla von
- Münter, Marc
- Wessalowski, Rüdiger
- Hartwig, Maite
- Sparber‐Sauer, Monika
- Schlegel, Paul‐Gerhardt
- Kramm, Christof M.
- Kontny, Udo
- Spriewald, Bernd
- Kegel, Thomas
- Bauer, Sebastian
- Kazanowska, Bernarda
- Niggli, Felix
- Ladenstein, Ruth
- Ljungman, Gustaf
- Jahnukainen, Kirsi
- Fuchs, Jörg
- Bielack, Stefan S.
- Klingebiel, Thomas
- Koscielniak, Ewa
DOIAbstract
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, e...
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