Soft Tissue Sarcoma Research

Results from a French clinical trial have identified what experts say should now be the recommended initial treatment of advanced leiomyosarcoma. In the trial, the combination of trabectedin (Yondelis) and doxorubicin improved survival by a median of 9 months.

Trial participants who stopped imatinib had a more rapid worsening of disease, a shorter time until resistance, and did not live as long as participants who continued the therapy uninterrupted.

Treatment with atezolizumab (Tecentriq) shrank tumors in about 40% of people with alveolar soft part sarcoma, including one complete response, new clinical trial findings show. Some people were later able to stop treatment without the cancer returning.

In a clinical trial, the drug nirogacestat shrank tumors in 40% of people with desmoid tumors. Treatment with nirogacestat also substantially improved progression-free survival, pain, and physical functioning, compared with patients treated with a placebo.

A clinical trial led by NCI has resulted in FDA approval of the immunotherapy drug atezolizumab (Tecentriq) to treat advanced alveolar soft part sarcoma.

An NCI study in mice has identified a drug combination that may help treat children with rhabdomyosarcoma. But one of the drugs, ganitumab, is no longer being made. Based on the study's promising results, the NCI researchers who led the study want to test the combination in humans.

The fusion protein that drives the growth and survival of some rhabdomyosarcoma tumors relies on the KDM4B enzyme, researchers have found. Treating mice with a KDM4B-blocking drug and chemotherapy nearly eliminated rhabdomyosarcoma tumors with the fusion protein.

For children and adults with advanced soft tissue sarcoma, adding pazopanib (Votrient) to chemotherapy and radiation before surgery may be a promising treatment option, early results from a clinical trial suggest.

For people with advanced AIDS-related Kaposi sarcoma in sub-Saharan Africa, results from a large clinical trial are expected to change treatment. In the trial, paclitaxel greatly improved outcomes compared with treatments typically used in the region.

Avapritinib (Ayvakit) has been approved for adults with gastrointestinal stromal tumors (GIST) whose tumors have an alteration in a portion of the PDGFRA gene called exon 18. The approval applies to those whose tumors cannot be removed with surgery or have spread.

Scientists may have pinpointed the cause of some gastrointestinal stromal tumors (GISTs), a rare cancer, according to a new NCI-funded study. However, the culprit isn’t a harmful genetic mutation, but another type of genetic change, what are called epigenetic alterations.

People with advanced alveolar soft part sarcoma (ASPS), a rare cancer, appear to benefit from a type of immunotherapy called an immune checkpoint inhibitor, according to results from a small clinical trial.

Interim results from a clinical trial for patients with desmoid tumors or aggressive fibromatosis (DT/DF) show that the drug sorafenib tosylate (Nexavar) extended progression-free survival compared with a placebo.

Two new targeted therapies have shown promise in patients with gastrointestinal stromal tumors (GIST) that have developed resistance to standard therapies.

The Food and Drug Administration (FDA) has granted accelerated approval to olaratumab (Lartruvo®) for the treatment of some patients with soft tissue sarcoma.

The FDA has approved eribulin mesylate for patients with liposarcoma whose cancers are advanced or cannot be removed by surgery and are no longer responding to anthracycline-based chemotherapy.