Evidence map›Paper›PMID 30652419›Full record

Trial reportCancer medicine2019

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

Monika Scheer, Christian Vokuhl, Bernd Blank, Erika Hallmen, Thekla von Kalle, Marc Münter, Rüdiger Wessalowski, Maite Hartwig, Monika Sparber-Sauer, Paul-Gerhardt Schlegel and 15 more

Registry-linked trialOpen access · goldAbstract readClinical Trial
In one paragraph

Trial report in Cancer medicine, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT07328425 (Phase II Study of Lurbinectedin and Irinotecan in Adult and Young Adult Patients With Advanced Desmoplastic Small Round Cell Tumor), which is not on this map. Cited by 25 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
25citing papers in PubMed, 1 pooled it
6.6field-weighted citation impact, top 3% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT07328425 phase2recruitingnot on this mapstarted 2025, after this paper: background citation

Phase II Study of Lurbinectedin and Irinotecan in Adult and Young Adult Patients With Advanced Desmoplastic Small Round Cell Tumor (DSRCT)

TypeinterventionalSponsorItalian Sarcoma GroupRan2025 to 2030Enrolled20ConditionsDesmoplastic Small Round Cell TumorArmsLurbinectedin, Irinotecan
3 · Its place in the literature

Who cites it

25 citing papers in PubMed, 1 synthesis or guideline pooled it, 57 citations in OpenAlex.

  1. Pooled it
  2. Phase II Study of ONC201 in Neuroendocrine Tumors including Pheochromocytoma-Paraganglioma and Desmoplastic Small Round Cell Tumor.Clinical cancer research : an official journal of the American Association for Cancer Research · 2022
    Trial
  3. Trial
  4. Review
  5. Review
  6. Article
  7. Article
  8. Article
  9. Article
  10. Article
  11. Review
  12. Article
  13. Small round cell sarcomas.Nature reviews. Disease primers · 2022
    Review
  14. Changing incidence and survival of desmoplastic small round cell tumor in the USA.Proceedings (Baylor University. Medical Center) · 2022
    Article
  15. Review
  16. Article
  17. Review
  18. Article
  19. Article
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

25 authors at 19 institutions in 6 countries.

Monika ScheerPediatrics 5, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.ORCID 0000-0002-0665-6268
Christian VokuhlKiel Peadiatric Tumour Registry, Department of Pediatric Pathology, University Hospital Kiel, Germany.
Bernd BlankPediatrics 5, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.
Erika HallmenPediatrics 5, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.
Thekla von KalleRadiologisches Institut, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.
Marc MünterRadiation Oncology, Klinikum Stuttgart, Stuttgart, Germany.
Rüdiger WessalowskiPediatric Oncology Clinic, Heinrich-Heine-University Düsseldorf, Düsseldorf, Germany.
Maite HartwigPediatric Hematology and Oncology, University Hamburg-Eppendorf, Hamburg, Germany.
Monika Sparber-SauerPediatrics 5, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.ORCID 0000-0001-9551-2399
Paul-Gerhardt SchlegelChildren's Hospital, Pediatric Oncology, University Würzburg, Würzburg, Germany.
Christof M KrammPediatric Hematology and Oncology, University Medical Center Göttingen, Göttingen, Germany.
Udo KontnyPediatric Hematology and Oncology, University Medical Center Aachen, Aachen, Germany.
Bernd SpriewaldInternal Medicine 5, University Hospital Erlangen, Erlangen, Germany.
Thomas KegelHematology/Oncology, University of Halle, Halle, Germany.
Sebastian BauerSarcoma Center, West German Cancer Center, University of Duisburg-Essen, Essen, Germany.
Bernarda KazanowskaDepartment of Pediatric Hematology/Oncology and BMT, University of Wroclaw, Wroclaw, Poland.
Felix NiggliPediatric Oncology, University of Zürich, Zürich, Switzerland.
Ruth LadensteinSt. Anna Kinderspital and St. Anna Kinderkrebsforschung e.V., Vienna, Austria.
Gustaf LjungmanDepartment of Women's and Children's Health, Uppsala University, Uppsala, Sweden.
Kirsi JahnukainenPediatrics, Helsinki University Central Hospital, Helsinki, Finland.
Jörg FuchsPediatric Surgery, University Hospital Tübingen, Tübingen, Germany.
Stefan S BielackPediatrics 5, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.
Thomas KlingebielDepartment for Children and Adolescents, University Hospital, Goethe-University Frankfurt (Main), Frankfurt, Germany.
Ewa KoscielniakPediatrics 5, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany.
Cooperative Weichteilsarkom Studiengruppe [CWS]
Olgahospital · DEChristian-Albrechts-Universität zu Kiel · DEGoethe University Frankfurt · DEHeinrich Heine University Düsseldorf · DEHelsinki University Hospital · FIKlinikum Stuttgart · DESt Anna Children's Hospital · ATUniversität Hamburg · DEUniversitätsklinikum Aachen · DEUniversitätsklinikum Erlangen · DEUniversitätsklinikum Würzburg · DEUniversitätsmedizin Göttingen · DEUniversity Children's Hospital Tübingen · DEUniversity Hospital in Halle · DEUniversity Hospital Münster · DEUniversity of Duisburg-Essen · DEUniversity of Wrocław · PLUniversity of Zurich · CHUppsala University · SE

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundTo evaluate optimal therapy and potential risk factors.

methodsData of DSRCT patients <40 years treated in prospective CWS trials 1997-2015 were analyzed.

resultsMedian 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.

conclusionPleural effusions, venous thrombosis, and CRP elevation were identified as potential risk factors. The VAIA scheme showed best outcome. Maintenance therapy should be investigated further.

Indexed as

Abdominal NeoplasmsAdolescentAdultAntineoplastic AgentsAntineoplastic Combined Chemotherapy ProtocolsChildCombined Modality TherapyC-Reactive ProteinDesmoplastic Small Round Cell TumorFemaleHumansMalePleural EffusionPrognosisRisk FactorsStem Cell TransplantationAntineoplastic AgentsC-Reactive ProteinC-reactive proteindesmoplastic small round cell tumormaintenance therapysoft tissue sarcomaTrousseau’s syndrome

Identifiers

PMID30652419
PMCPMC6382921
OpenAlexW2910361966

What OpenQuestion holds

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LicenceCC BY
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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.