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

Zitieren Sie bitte immer diese URN: urn:nbn:de:bvb:20-opus-228444
  • 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%) elevatedBackground 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.zeige mehrzeige weniger

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Metadaten
Autor(en): 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:bvb:20-opus-228444
Dokumentart:Artikel / Aufsatz in einer Zeitschrift
Institute der Universität:Medizinische Fakultät / Kinderklinik und Poliklinik
Sprache der Veröffentlichung:Englisch
Titel des übergeordneten Werkes / der Zeitschrift (Englisch):Cancer Medicine
Erscheinungsjahr:2019
Band / Jahrgang:8
Heft / Ausgabe:2
Seitenangabe:527-545
Originalveröffentlichung / Quelle:Cancer Med. 2019;8:527–542.
DOI:https://doi.org/10.1002/cam4.1940
Allgemeine fachliche Zuordnung (DDC-Klassifikation):6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Freie Schlagwort(e):C-reactive protein; Trousseau's syndrome; desmoplastic small round cell tumor; maintenance therapy; soft tissue sarcoma
Datum der Freischaltung:30.11.2021
Lizenz (Deutsch):License LogoCC BY: Creative-Commons-Lizenz: Namensnennung 4.0 International