Chordomas and chondrosarcomas are rare but devastating neoplasms that are characterized by chemoradiation resistance. For both tumors, surgical resection is the cornerstone of management. Immunotherapy agents are increasingly improving outcomes in multiple cancer subtypes and are being explored in chordoma and chondrosarcoma alike. In chordoma, brachyury has been identified as a prominent biomarker and potential molecular immunotherapy target as well as PD-1 inhibition. While studies on immunotherapy in chondrosarcoma are sparse, there is emerging evidence and ongoing clinical trials for PD-1 as well as IDH inhibitors. This review highlights potential biomarkers and targets for immunotherapy in chordoma and chondrosarcoma, as well as current clinical evidence and ongoing trials.
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| http://dx.doi.org/10.3390/cancers13102408 | DOI Listing |
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Study Design: Systematic review.
Objectives: The objective of this review paper was to summarize targeted molecular therapy options for spinal chordoma and chondrosarcoma, and to provide an update on the relevant clinical trials open for recruitment.
Methods: A systematic review of the current literature was performed, according to PRISMA guidelines, to summarize the latest developments in non-surgical molecular treatment options for low grade malignant primary spinal tumours.
Liquid Biopsy for Spinal Tumors: On the Frontiers of Clinical Application.
Global Spine J
January 2025
Combined Neurosurgical and Orthopedic Spine Program, Department of Orthopedics Surgery, University of British Columbia, Vancouver, BC, Canada.
Study Design: Narrative review.
Objectives: This article aims to provide a narrative review of the current state of research for liquid biopsy in spinal tumors and to discuss the potential application of liquid biopsy in the clinical management of patients with spinal tumors.
Methods: A comprehensive review of the literature was performed using PubMed, Google Scholar, Medline, Embase and Cochrane databases, and the review was limited to articles of English language.
Developmental gene expression in skull-base chordomas and chondrosarcomas.
J Neurooncol
December 2024
Department of Neurosurgery, Maastricht University Medical Center, P. Debyelaan 25, 6202 AZ, Maastricht, The Netherlands.
Purpose: Chordomas are malignant tumors of the axial spine and skull base, and they are notorious for their poor treatment response. Differentiating these tumors from comparatively less malignant chondrosarcomas is crucial for treatment and prognostication. Both tumor types differ in their developmental origin.
View Article and Find Full Text PDFChronic hearing loss turns out being a calcified chondroid mesenchymal neoplasm with FN1::FGFR2 fusion.
Eur Arch Otorhinolaryngol
October 2024
Barmherzige Brüder Klinikum St. Elisabeth Straubing, Klinik für Hals-Nasen-Ohren-Heilkunde mit Kopf-Hals- und plastischer Gesichtschirurgie, Straubing, Germany.
Article Synopsis
- * The tumor, identified as a "calcified chondroid mesenchymal neoplasm" (CCMN) through molecular analysis, was surgically removed, showing extensive changes indicative of its aggressive nature.
- * Follow-up imaging for over two years showed no residual tumor, but ongoing monitoring is crucial due to potential local recurrences; targeted therapies may be considered for any future recurrences involving the FGFR2 fusion.
Spinal chordoma and chondrosarcoma treatment experiences - a 20-year retrospective study from databases of two medical centers.
Sci Rep
October 2024
Divison of Neurosurgery, Departmet of Surgery, National Taiwan University Hospital, No.7, Chung Shan S. Rd. (Zhongshan S. Rd.), Zhongzheng Dist., Taipei, 100225, Taiwan (R.O.C.).
Article Synopsis
- The study analyzed 48 patients with spinal chordoma and chondrosarcoma treated from 2001 to 2023, focusing on the type of surgical margins achieved during tumor removal.
- Patients with negative or microscopically positive margins (R0/R1) had a significantly better overall survival (OS) and progression-free survival (PFS) compared to those with macroscopically positive margins (R2), implying that margin status is crucial for treatment outcome.
- Radiation therapy (RT) did not enhance OS or PFS in either group, leading to the conclusion that en bloc resection surgery with a proper margin is the preferred treatment approach, while radiation may be an option for those unable to have surgery
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