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Review
. 2023 Dec 11;15(24):5800.
doi: 10.3390/cancers15245800.

Chordoma: A Comprehensive Systematic Review of Clinical Trials

Sonja Chen  1 , Ruben Ulloa  2 , Justin Soffer  3 , Roberto J Alcazar-Felix  1 , Carl H Snyderman  4 , Paul A Gardner  5 , Vijay A Patel  6   7 , Sean P Polster  1
Affiliations
Review

Chordoma: A Comprehensive Systematic Review of Clinical Trials

Sonja Chen et al. Cancers (Basel). .

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.

Keywords: chordoma; clinical trials; endonasal; endoscopic; notochord tumor; skull base; systematic review.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 5
Figure 5
Molecular targets related to chordoma.
Figure 1
Figure 1
(a) Irregular epithelial cell clusters and interlacing strands of vacuolated physaliphorous cells in a mucoid matrix in a case of clival chordoma (H&E 4×, 20×). (b) Immunopositivity for Brachyury in chordoma (IHC, 20×).
Figure 2
Figure 2
(a) T2-weighted MRI coronal sequence demonstrating the characteristic hallmarks of a clival chordoma, T2 hyperintensity within the skull base lesion. (b) T1-weighted MRI post-contrast sagittal sequence demonstrating anatomical boundaries of a clival chordoma with ventral brainstem compression. (c) T1-weighted MRI post-contrast sagittal sequence demonstrating gross total resection via endoscopic endonasal approach of clival chordoma.
Figure 2
Figure 2
(a) T2-weighted MRI coronal sequence demonstrating the characteristic hallmarks of a clival chordoma, T2 hyperintensity within the skull base lesion. (b) T1-weighted MRI post-contrast sagittal sequence demonstrating anatomical boundaries of a clival chordoma with ventral brainstem compression. (c) T1-weighted MRI post-contrast sagittal sequence demonstrating gross total resection via endoscopic endonasal approach of clival chordoma.
Figure 3
Figure 3
PRISMA flowchart of the inclusion and exclusion of chordoma clinical trials (1999–2022).
Figure 4
Figure 4
(a)Therapeutic clinical trials for chordoma (1999–2022). (b) Observational clinical trials for chordoma (1999–2022).
Figure 4
Figure 4
(a)Therapeutic clinical trials for chordoma (1999–2022). (b) Observational clinical trials for chordoma (1999–2022).
See this image and copyright information in PMC

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