ReviewSarcoma2017
Desmoplastic Small Round Blue Cell Tumor: A Review of Treatment and Potential Therapeutic Genomic Alterations.
Review in Sarcoma, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT07695311 (Precision Analysis of Fusion Genes in Ewing Sarcoma), which is not on this map. Cited by 33 papers, 1 of them a synthesis that pooled 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.
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.
Precision Analysis of Fusion Genes in Ewing Sarcoma (ES) and Desmoplastic Small Round Cell Tumor (DSRCT), Then EWSR1 (Ewing Sarcoma Gene Breakpoint Region 1 Gene) Immunotherapy Without or With Anti-CTLA-4 (Botensilimab) and Anti-PD1 (Balstilimab)
Who cites it
33 citing papers in PubMed, 1 synthesis or guideline pooled it, 64 citations in OpenAlex.
- Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives.Frontiers in oncology · 2021Pooled it
- Desmoplastic small round cell tumor of the kidney with bone metastasis: A case report.Radiology case reports · 2026Article
- Review
- Desmoplastic Small Round Blue Cell Tumor in a Young Adult Man: A Rare Case of Abdominal Sarcoma.The American journal of case reports · 2026Article
- Targeting developmental vulnerabilities in childhood sarcomas.Cancer metastasis reviews · 2025Review
- Multiple Desmoplastic Small Round Cell Tumor in the Intestine: A Case Report.Surgical case reports · 2025Article
- Article
- Single-cell multiomics profiling reveals heterogeneous transcriptional programs and microenvironment in DSRCTs.Cell reports. Medicine · 2024Article
- Article
- Article
- Desmoplastic small round cell tumor: from genomics to targets, potential paths to future therapeutics.Frontiers in cell and developmental biology · 2024Review
- Article
- Case report: An unusual presentation of intra-abdominal desmoplastic small round cell tumor.Frontiers in oncology · 2024Article
- Effectiveness of irinotecan plus trabectedin on a desmoplastic small round cell tumor patient-derived xenograft.Disease models & mechanisms · 2023Article
- The androgen receptor is a therapeutic target in desmoplastic small round cell sarcoma.Nature communications · 2022Article
- Multi-site desmoplastic small round cell tumors are genetically related and immune-cold.NPJ precision oncology · 2022Article
- Desmoplastic small round cell tumor cancer stem cell-like cells resist chemotherapy but remain dependent on the EWSR1-WT1 oncoprotein.Frontiers in cell and developmental biology · 2022Article
- Targeting the FAK-Src Complex in Desmoplastic Small Round Cell Tumors, Ewing Sarcoma, and Rhabdomyosarcoma.Sarcoma · 2022Article
- Lysine specific demethylase 1 is a molecular driver and therapeutic target in sarcoma.Frontiers in oncology · 2022Review
- EWSR1-WT1 Target Genes and Therapeutic Options Identified in a Novel DSRCT In Vitro Model.Cancers · 2021Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
7 authors at 7 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Desmoplastic small round blue cell tumors (DSRCTs) originate from a cell with multilineage potential. A molecular hallmark of DSRCT is the EWS-WT1 reciprocal translocation. Ewing sarcoma and DSRCT are treated similarly due to similar oncogene activation pathways, and DSRCT has been represented in very limited numbers in sarcoma studies. Despite aggressive therapy, median survival ranges from 17 to 25 months, and 5-year survival rates remain around 15%, with higher survival reported among those undergoing removal of at least 90% of tumor in the absence of extraperitoneal metastasis. Almost 100% of these tumors contain t(11;22) (p13;q12) translocation, and it is likely that EWS-WT1 functions as a transcription factor possibly through WT1 targets. While there is no standard protocol for this aggressive disease, treatment usually includes the neoadjuvant HD P6 regimen (high-dose cyclophosphamide, doxorubicin, and vincristine (HD-CAV) alternating with ifosfamide and etoposide (IE) chemotherapy combined with aggressively attempted R0 resection). We aimed to review the molecular characteristics of DSRCTs to explore therapeutic opportunities for this extremely rare and aggressive cancer type.
Identifiers
What OpenQuestion holds
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.