Trial reportPediatric blood & cancer2021
Suboptimal outcome for patients with biliary rhabdomyosarcoma treated on low-risk clinical trials: A report from the Children's Oncology Group.
Trial report in Pediatric blood & cancer, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
11 citing papers in PubMed, 1 synthesis or guideline pooled it, 17 citations in OpenAlex.
- Biliary Rhabdomyosarcoma in Pediatric Patients: A Systematic Review and Meta-Analysis of Individual Patient Data.Frontiers in oncology · 2021Pooled it
- Review
- Multimodality imaging in the diagnosis of biliary rhabdomyosarcoma in a young child: a case report.Frontiers in pediatrics · 2026Article
- Surgical management of pediatric biliary rhabdomyosarcoma: Importance of differential diagnosis.Hepatology forum · 2025Article
- Rhabdomyosarcoma of the Biliary Tract in Children: Analysis of Single Center Experience.Cancers · 2024Article
- Article
- Rhabdomyosarcoma of the biliary tract in a child: a case report.Frontiers in pediatrics · 2024Article
- Review on Pediatric Malignant Focal Liver Lesions with Imaging Evaluation: Part II.Diagnostics (Basel, Switzerland) · 2023Review
- Review
- Clinical group and modified TNM stage for rhabdomyosarcoma: A review from the Children's Oncology Group.Pediatric blood & cancer · 2022Review
- An update on rhabdomyosarcoma risk stratification and the rationale for current and future Children's Oncology Group clinical trials.Pediatric blood & cancer · 2022Review
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Authors and funding
7 authors at 7 institutions in 1 country.
Funding
Abstract
backgroundBiliary rhabdomyosarcoma (RMS) is the most common biliary tumor in children. The biliary tract is classified as a favorable primary site. Therefore, patients with localized biliary RMS were included in two consecutive low-risk studies, D9602 and ARST0331, by the Children's Oncology Group (COG). The outcome for these patients treated with low-risk therapy has not been reported. PROCEDURE: Patients with biliary RMS enrolled on COG low-risk trials D9602 or ARST0331 were analyzed. All patients received systemic chemotherapy and those with Group II (microscopic residual) or Group III (macroscopic residual) disease received 36-50.4 Gy adjuvant radiotherapy (RT). Delayed primary excision (DPE) was allowed on both studies.
resultsSeventeen patients with biliary RMS were treated on D9602 (n = 7) or ARST0331 (n = 10). Median age was 3.5 years (range 1.7-10.3). Ten (59%) patients had tumors >5 cm and 14 (82%) had Group III disease. Fifteen (88%) patients received RT. The 5-year event-free survival (EFS) and overall survival (OS) were 70.6% (95% confidence interval [CI]: 46.9-94.3%) and 76.5% (95% CI: 54.6-98.4%), respectively. The majority of patients (80%) who received RT did not have disease recurrence while both patients who did not receive RT had local relapse. Five (36%) of 14 patients with Group III disease underwent DPE; two experienced a local relapse. In the nine patients without DPE, two developed local relapse.
conclusionsPatients with localized biliary RMS treated on low-risk studies had suboptimal outcomes. These patients may benefit from therapy on intermediate-risk studies.
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