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

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 (2019)

Publication Type: Journal article

Publication year: 2019

Journal

Book Volume: 8

Pages Range: 527-542

Journal Issue: 2

DOI: 10.1002/cam4.1940

Abstract

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.

Involved external institutions

Universitätsklinikum Göttingen Germany (DE) Klinikum Stuttgart Germany (DE) Heinrich-Heine-Universität Düsseldorf Germany (DE) Universitätsklinikum Aachen (UKA) Germany (DE) Universitätsklinikum Hamburg-Eppendorf (UKE) Germany (DE) Julius-Maximilians-Universität Würzburg Germany (DE) St. Anna Kinderkrebsforschung Austria (AT) Universität Duisburg-Essen (UDE) Germany (DE) Goethe-Universität Frankfurt am Main Germany (DE) Helsingin yliopisto / University of Helsinki Finland (FI) Martin-Luther-Universität Halle-Wittenberg (MLU) Germany (DE) Eberhard Karls Universität Tübingen Germany (DE) University of Zurich / Universität Zürich (UZH) Switzerland (CH) University of Wroclaw / Uniwersytet Wrocławski Poland (PL) Universitätsklinikum Tübingen Germany (DE) Universitätsklinikum Schleswig-Holstein (UKSH) Germany (DE) Uppsala University Sweden (SE)

How to cite

APA:

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. https://dx.doi.org/10.1002/cam4.1940

MLA:

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

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