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Clinical Trial
. 2019 Feb;8(2):527-542.
doi: 10.1002/cam4.1940. Epub 2019 Jan 16.

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

Monika Scheer  1 , Christian Vokuhl  2 , Bernd Blank  1 , Erika Hallmen  1 , Thekla von Kalle  3 , Marc Münter  4 , Rüdiger Wessalowski  5 , Maite Hartwig  6 , Monika Sparber-Sauer  1 , Paul-Gerhardt Schlegel  7 , Christof M Kramm  8 , Udo Kontny  9 , Bernd Spriewald  10 , Thomas Kegel  11 , Sebastian Bauer  12 , Bernarda Kazanowska  13 , Felix Niggli  14 , Ruth Ladenstein  15 , Gustaf Ljungman  16 , Kirsi Jahnukainen  17 , Jörg Fuchs  18 , Stefan S Bielack  1   19 , Thomas Klingebiel  20 , Ewa Koscielniak  1   21 ; Cooperative Weichteilsarkom Studiengruppe [CWS]
Affiliations
Clinical Trial

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

Monika Scheer et al. Cancer Med. 2019 Feb.

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.

Keywords: C-reactive protein; Trousseau’s syndrome; desmoplastic small round cell tumor; maintenance therapy; soft tissue sarcoma.

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Figures

Figure 1
Figure 1
Event‐free and overall survival probability of 60 DSRCT patients. Event‐free survival probability according to the tumor distribution, the existence of effusions, thrombosis, elevated pretreatment CRP‐value, and the conducted first‐line chemotherapeutic regimens and best surgical result at any time in first‐line therapy
Figure 2
Figure 2
Flow diagram of evaluated patients
Figure 3
Figure 3
The effect of additional maintenance therapy after the end of intensive chemotherapy evaluated in 25 patients who achieved a first complete remission
See this image and copyright information in PMC

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