ArticleRare tumors2016
Clinical Characteristics and Outcomes of Pediatric Patients with Desmoplastic Small Round Cell Tumor.
Article in Rare tumors, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers, 1 of them a synthesis that pooled it.
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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.
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Who cites it
16 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Extracellular Vesicles: A New Source of Biomarkers in Pediatric Solid Tumors? A Systematic Review.Frontiers in oncology · 2022Pooled it
- Paediatric therapeutic development workshop on desmoplastic small round cell tumours.British journal of cancer · 2026Review
- Prevalence of locoregional and distant lymph node metastases in children and adolescents/young adults with soft tissue sarcomas: a Bayesian meta-analysis of proportions.EClinicalMedicine · 2025Article
- Prognostic factors of desmoplastic small round cell tumor in children and adolescents: a population-based study.Pediatric surgery international · 2025Article
- Article
- A Review of the Use of Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Malignancy in Pediatric Patients.Cancers · 2023Review
- Small round cell sarcomas.Nature reviews. Disease primers · 2022Review
- Hyperthermic intraperitoneal chemotherapy (HIPEC) as another treatment modality for desmoplastic round cell tumour patients: first paediatric experience from UK.BMJ case reports · 2021Article
- The addition of autologous stem cell transplantation to neoadjuvant chemotherapy, radiation, and HIPEC for patients with unresectable desmoplastic small round cell tumor: a single center case series.International journal of surgery. Oncology · 2020Article
- The use of interval-compressed chemotherapy with the addition of vincristine, irinotecan, and temozolomide for pediatric patients with newly diagnosed desmoplastic small round cell tumor.Pediatric blood & cancer · 2020Article
- Olaparib and temozolomide in desmoplastic small round cell tumors: a promising combination in vitro and in vivo.Journal of cancer research and clinical oncology · 2020Article
- A novel image-based system for risk stratification in patients with desmoplastic small round cell tumor.Journal of pediatric surgery · 2020Article
- Systemic therapy in pediatric-type soft-tissue sarcoma.Current oncology (Toronto, Ont.) · 2020Review
- Mini-Review on Targeted Treatment of Desmoplastic Small Round Cell Tumor.Frontiers in oncology · 2020Review
- Sinonasal desmoplastic small round cell tumor: a case report and review of the literature.BMC cancer · 2019Review
- Expression and clinical association of programmed cell death-1, programmed death-ligand-1 and CD8Oncotarget · 2017Article
Corrections and comments
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Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Desmoplastic small round cell tumor (DSRCT) is a rare malignancy that typically affects pediatric and young adult patients. There are limited data on the clinical features of pediatric DSRCT. We selected patients aged 0-21 years reported to the Surveillance, Epidemiology and End Results Program from 1991-2011. We estimated overall survival using Kaplan-Meier approaches and compared outcomes using the log rank test. The median age of the 95 pediatric patients was 15.3 years (range: 0-21). The majority of tumors originated in the abdomen and pelvis (84.4%) and the majority of patients had distant metastasis (72.6%). A minority of patients received radiation (34%). Overall survival at 5 years was poor (18.1%; 95% confidence interval 10.1-27.9%). Radiation therapy was associated with superior survival. Pediatric patients with DSRCT have significant disease burden. Outcomes for children are poor, though patients selected for radiation appear to have improved survival.
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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.