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Chordoma: A Comprehensive Systematic Review of Clinical Trials
Sonja Chen1, Ruben Ulloa2, Justin Soffer3
1Department of Neurosurgery, University of Chicago, Chicago, IL 60637, USA.
Abstract:
This systematic review aims to characterize ongoing clinical trials and therapeutic treatment options for chordoma, a rare notochordal remnant tumor that primarily affects the cranial base, mobile spine, and sacrum. While radical surgical resection remains the cornerstone for chordoma management, unique technical challenges posed by its proximity to critical neurovascular structures confer a tendency towards disease recurrence which often requires additional treatment modalities. In an attempt to better understand the current treatment landscape, a systematic review was designed to identify clinical trials directed at chordoma. A total of 108 chordoma trials were identified from four clinical trial databases; fifty-one trials were included in the final analysis, of which only 14 were designated as completed (27.5%). Aggregate data suggests most chordoma interventions are repurposed from other neoplasms that share common molecular pathways, with a recent emphasis on combination therapeutics within and across drug classes. Naturally, the publication and dissemination of clinical trial results remain a concern (n = 4, 28.6%), highlighting the need for enhanced reporting and transparency measures. Active clinical trial efforts are quite promising, with a renewed focus on novel biotherapeutic targets and deciphering the natural history, as well as survivorship of this complex disease.
Insights
This review analyzes chordoma clinical trials, finding many treatments are repurposed from other cancers. Enhanced reporting is needed for these rare tumors, but new therapies show promise.
Area of Science:
- Oncology
- Rare Cancers
- Clinical Trial Analysis
Background:
- Chordoma is a rare bone tumor originating from notochordal remnants, often affecting the skull base, spine, and sacrum.
- Surgical resection is primary treatment but recurrence is common due to proximity to vital structures.
- Understanding current therapeutic options and clinical trial landscape is crucial for improving chordoma management.
Purpose of the Study:
- To systematically review and characterize ongoing clinical trials for chordoma.
- To identify and analyze therapeutic treatment options currently under investigation.
- To assess the status and outcomes of completed chordoma clinical trials.
Main Methods:
- Systematic review of clinical trials identified from four major databases.
- Inclusion criteria focused on trials directly addressing chordoma.
- Analysis of trial status, completion rates, and therapeutic strategies employed.
Main Results:
- 108 chordoma trials were identified, with 51 included in the final analysis.
- Only 27.5% of the analyzed trials were completed, indicating a slow progression.
- Most interventions are repurposed from other cancers, with a trend towards combination therapies.
- Publication rates for completed trials were low (28.6%), highlighting transparency issues.
Conclusions:
- Chordoma research is active, with promising new biotherapeutic targets and a focus on disease natural history.
- Repurposed therapies and combination treatments are common, but trial completion and reporting need improvement.
- Enhanced transparency and reporting of clinical trial results are essential for advancing chordoma treatment.
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