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Updated: Feb 17, 2026

Three-Dimensional Bone Extracellular Matrix Model for Osteosarcoma
Published on: April 12, 2019
Osteosarcoma, Chondrosarcoma, and Chordoma
Jeremy S Whelan1, Lara E Davis1
1Jeremy S. Whelan, University College London Hospitals NHS Foundation Trust, London, United Kingdom; and Lara E. Davis, Knight Cancer Institute, Oregon Health & Science University, Portland, OR.
Abstract:
Osteosarcoma (OS), chondrosarcoma, and chordoma are characterized by multiple challenges to the investigator, clinician, and patient. One consequence of their rarity among sarcomas, as well as their biologic and clinical heterogeneity, is that management guidelines are inadequate to inform the range of individual patient-treatment decisions from diagnosis, approaches to surgery, chemotherapy, radiotherapy, treatment of recurrence, palliative care, and quality of survivorship. Of high-grade sarcomas, OSs are among the most curable, with more than two-thirds of patients with localized disease likely to achieve long-term survival. Neoadjuvant chemotherapy comprising cisplatin, doxorubicin, and methotrexate with intercalated surgery is the standard of care for resectable OS in those younger than 40 years. Outcomes for OS presenting with unresectable metastases or recurrent disease, or in those older than 40 years are generally poor. Overall results have improved little for all patients with OS, and new treatments are needed. Surgical resection remains the cornerstone of management for chondrosarcoma and chordoma. However, the application of new biologic insights to therapeutic development indicates that improved treatments may soon be routine for patients with chondrosarcoma and chordoma for whom surgery alone is inadequate. For all these uncommon diseases, patients should be offered specialist expert care delivered by experienced multidisciplinary teams in high-volume centers.
Insights
Osteosarcoma, chondrosarcoma, and chordoma present management challenges due to rarity and heterogeneity. New treatments are needed, especially for advanced osteosarcoma, while improved therapies are emerging for chondrosarcoma and chordoma.
Area of Science:
- Orthopedic Oncology
- Surgical Oncology
- Medical Oncology
Background:
- Osteosarcoma, chondrosarcoma, and chordoma are rare bone cancers with significant management challenges.
- Their rarity and heterogeneity result in inadequate guidelines for diagnosis, treatment, and survivorship.
- Current standard care for localized osteosarcoma (OS) offers good prognosis in younger patients, but outcomes are poor for metastatic or recurrent disease and older patients.
Purpose of the Study:
- To review the current management challenges and therapeutic landscape for osteosarcoma, chondrosarcoma, and chordoma.
- To highlight the need for improved treatment strategies and specialized care for these rare bone tumors.
- To discuss the potential of new biologic insights for future therapeutic development.
Main Methods:
- Review of existing literature and clinical guidelines for osteosarcoma, chondrosarcoma, and chordoma.
- Analysis of current treatment standards, including chemotherapy, surgery, and radiotherapy.
- Discussion of prognostic factors and outcomes based on disease stage and patient age.
Main Results:
- Osteosarcoma shows high curability in localized disease for younger patients with standard neoadjuvant chemotherapy and surgery.
- Outcomes for unresectable, recurrent, or older adult osteosarcoma patients remain poor, indicating a need for novel therapies.
- Surgical resection is primary for chondrosarcoma and chordoma, with emerging biologic insights promising improved future treatments.
Conclusions:
- Despite advances, significant unmet needs persist for osteosarcoma, chondrosarcoma, and chordoma management.
- Multidisciplinary expert care in high-volume centers is crucial for optimizing outcomes in these rare bone tumors.
- Continued research into biologic insights is essential for developing more effective treatments for chondrosarcoma and chordoma.
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