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Updated: Feb 13, 2026

Establishment of a Primary Culture of Patient-derived Soft Tissue Sarcoma
Published on: April 11, 2018
Current Molecular Targeted Therapies for Bone and Soft Tissue Sarcomas
Kenji Nakano1, Shunji Takahashi2
1Department of Medical Oncology, Cancer Institute Hospital of Japanese Foundation for Cancer Research, 3-8-31 Ariake, Koto, Tokyo 135-8550, Japan. kenji.nakano@jfcr.or.jp.
Abstract:
Systemic treatment options for bone and soft tissue sarcomas remained unchanged until the 2000s. These cancers presented challenges in new drug development partly because of their rarity and heterogeneity. Many new molecular targeting drugs have been tried in the 2010s, and some were approved for bone and soft tissue sarcoma. As one of the first molecular targeted drugs approved for solid malignant tumors, imatinib's approval as a treatment for gastrointestinal stromal tumors (GISTs) has been a great achievement. Following imatinib, other tyrosine kinase inhibitors (TKIs) have been approved for GISTs such as sunitinib and regorafenib, and pazopanib was approved for non-GIST soft tissue sarcomas. Olaratumab, the monoclonal antibody that targets platelet-derived growth factor receptor (PDGFR)-α, was shown to extend the overall survival of soft tissue sarcoma patients and was approved in 2016 in the U.S. as a breakthrough therapy. For bone tumors, new drugs are limited to denosumab, a receptor activator of nuclear factor κB ligand (RANKL) inhibitor, for treating giant cell tumors of bone. In this review, we explain and summarize the current molecular targeting therapies approved and in development for bone and soft tissue sarcomas.
Insights
Molecular targeted therapies have advanced bone and soft tissue sarcoma treatment since the 2000s. Approved drugs like imatinib and denosumab offer new options for these rare cancers.
Area of Science:
- Oncology
- Pharmacology
- Cancer Research
Background:
- Systemic treatment for bone and soft tissue sarcomas saw limited progress until the 2000s.
- Rarity and heterogeneity of these cancers posed challenges for drug development.
- The 2010s marked a shift with the introduction and approval of novel molecularly targeted drugs.
Purpose of the Study:
- To review and summarize current molecularly targeted therapies approved for bone and soft tissue sarcomas.
- To discuss molecularly targeted therapies currently in development for these conditions.
- To highlight key advancements in systemic treatment for sarcomas.
Main Methods:
- Literature review of approved and investigational molecularly targeted therapies.
- Summary of drug approvals and their indications for bone and soft tissue sarcomas.
- Analysis of drug mechanisms, including tyrosine kinase inhibitors and monoclonal antibodies.
Main Results:
- Imatinib approval for gastrointestinal stromal tumors (GISTs) was a significant early success.
- Tyrosine kinase inhibitors (TKIs) like sunitinib and regorafenib are approved for GISTs.
- Pazopanib for non-GIST soft tissue sarcomas and denosumab for giant cell tumors of bone represent other key approvals.
- Olaratumab, a PDGFR-α inhibitor, demonstrated improved survival in soft tissue sarcoma patients.
Conclusions:
- Molecularly targeted therapies have significantly expanded systemic treatment options for sarcomas.
- Ongoing research and development continue to yield new therapeutic strategies.
- Targeted drug approvals offer improved outcomes for patients with bone and soft tissue sarcomas.
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