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

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http://hdl.handle.net/10138/300249

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Cooperative Weichteilsarkom , Scheer , M , Vokuhl , C , Blank , B , Jahnukainen , K & Koscielniak , E 2019 , ' Desmoplastic small round cell tumors : Multimodality treatment and new risk factors ' , Cancer medicine , vol. 8 , no. 2 , pp. 527-542 . https://doi.org/10.1002/cam4.1940

Title: Desmoplastic small round cell tumors : Multimodality treatment and new risk factors
Author: Cooperative Weichteilsarkom; Scheer, Monika; Vokuhl, Christian; Blank, Bernd; Jahnukainen, Kirsi; Koscielniak, Ewa
Contributor: University of Helsinki, Children's Hospital
Date: 2019-02
Language: eng
Number of pages: 16
Belongs to series: Cancer medicine
ISSN: 2045-7634
URI: http://hdl.handle.net/10138/300249
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.
Subject: C-reactive protein
desmoplastic small round cell tumor
maintenance therapy
soft tissue sarcoma
Trousseau's syndrome
HIGH-DOSE CHEMOTHERAPY
SOFT-TISSUE SARCOMA
DISSEMINATED INTRAVASCULAR COAGULATION
VENOUS THROMBOEMBOLISM
INTERNATIONAL SOCIETY
RADIATION-THERAPY
CLINICAL ACTIVITY
YOUNG-ADULTS
CHILDREN
CANCER
3122 Cancers
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