The OncLive Sarcomas/TGCT condition center page is a comprehensive resource for clinical news and expert insights on tenosynovial giant cell tumor (TGCT), soft tissue sarcoma, gastrointestinal stromal tumor, Ewing sarcoma, and more. This page features news articles, interviews in written and video format, and podcasts that focus on unmet needs, treatment advances, and ongoing research in sarcomas and TGCT.
Ripretinib Does Not Provide Superior PFS Benefit Over Sunitinib in Second-line GIST, But Shows Better Tolerability
Ripretinib did not result in a significant improvement in progression-free survival over sunitinib in patients with gastrointestinal stromal tumor who were previously treated with imatinib, missing the primary end point of the phase 3 INTRIGUE trial.
Ifosfamide Improves Survival vs Topotecan/Cyclophosphamide in R/R Ewing Sarcoma
Ifosfamide induced small improvements in terms of prolonging event-free survival and overall survival compared with topotecan plus cyclophosphamide in patients with relapsed/refractory Ewing sarcoma.
Nirogacestat Significantly Improves PFS in Progressing Desmoid TumorsMay 24th 2022
Nirogacestat was found to significantly improve progression-free survival over placebo when used in the treatment of adult patients with progressing desmoid tumors, meeting the primary end point of the phase 3 DeFi trial.
FDA Accepts NDA Resubmission for Pedmark in Prevention of Platinum-Induced Ototoxicity in Solid TumorsApril 28th 2022
The FDA has accepted for filing the new drug application resubmission for a unique formulation of sodium thiosulfate for the prevention of platinum-induced ototoxicity in patients between the ages of 1 month and 18 years who have localized, nonmetastatic, solid tumors.
NDA Resubmitted to FDA for Pedmark in Prevention of Platinum-Induced Ototoxicity in Solid TumorsMarch 24th 2022
A new drug application for a unique formulation of sodium thiosulfate was resubmitted to the FDA for the second time for the prevention of platinum-induced ototoxicity in patients between the ages of 1 month and 18 years who have localized, nonmetastatic, solid tumors.
Ultra-Rare Sarcoma Subgroup Sees First Treatment OptionMarch 7th 2022
Nab-sirolimus became a treatment option for a rare, aggressive soft-tissue sarcoma—malignant perivascular epithelioid cell tumor—following encouraging data in the first prospective clinical trial for this patient population.
NCCN Guidelines List Ripretinib as Preferred Fourth-Line Option in Gastrointestinal Stromal TumorFebruary 25th 2022
Version 1.2022 of the National Comprehensive Cancer Network guidelines now include ripretinib as a category 1 fourth-line treatment option for patients with advanced gastrointestinal stromal tumor and as an additional TKI for consideration prior to surgery.
Investigators Explore Metformin to Prevent Cancers in Li-Fraumeni Syndrome CarriersFebruary 22nd 2022
Individuals with Li-Fraumeni Syndrome, a rare hereditary condition, are at increased risk for a wide spectrum of malignancies, including breast cancer and sarcomas. Investigators are working to determine whether metformin, a drug indicated for diabetes and infertility caused by polycystic ovarian syndrome, can prevent cancers in LFS carriers.
Collaborations With Orthopedic Surgeons, Interventional Radiologists Key to Novel Approaches in Metastatic Bone CancerFebruary 15th 2022
R. Lor Randall, MD, discusses the importance of collaborations between orthopedic surgeons and interventional radiologists, and how they are beginning to pick up steam in the United States for patients with metastatic bone cancer.
Brachytherapy Represents a Potential Strategy in AYA Patients With RhabdomyosarcomaFebruary 9th 2022
Although brachytherapy is rarely utilized as a treatment for pediatric and adolescent/young adult patients in the United States, AYA patients with rhabdomyosarcoma treated with BT had favorable survival outcomes.
Imatinib/Binimetinib Combo Shows Efficacy With Manageable Safety in Treatment-Naïve Advanced GISTFebruary 8th 2022
The combination of the TKI inhibitor imatinib and the MEK inhibitor binimetinib elicited encouraging responses in patients with treatment-naïve advanced gastrointestinal stromal tumors.
Afami-cel Shows Clinical Benefit in Synovial Sarcoma and Myxoid/Round Cell Liposarcoma
Brian A. Van Tine, MD, PhD, sheds light on the promise of SPEAR T-cell therapy in patients with synovial sarcoma and MRCLS, the safety and efficacy observed with afami-cel in the SPEARHEAD-1 trial, and next steps for research.
FDA Grants Rare Pediatric Disease Designation to Novel Radiopharmaceutical for OsteosarcomaFebruary 2nd 2022
The FDA has granted a rare pediatric disease designation to the bone-targeting radiopharmaceutical, Samarium-153-DOTMP, for use as a potential therapeutic option for patients with osteosarcoma.
Gender, Racial, and Socioeconomic Status Disparities Linked With Incidence of Metastatic Bone DiseaseJanuary 26th 2022
R. Lor Randall, MD, speaks to the findings from a report that said multiple sex-related, racial/ethnic, and socioeconomic status disparities are associated with an increased incidence of metastatic bone disease originating from cancers in the prostate, renal, colon, lung, and breast.
Alpha DaRT Elicits Complete Responses in Malignant Skin and Soft Tissue CancersJanuary 11th 2022
The diffusing alpha-emitter radiation therapy, Alpha DaRT, was found to elicit complete responses per RECIST v1.1 criteria in 10 patients with malignant skin and soft tissue cancers who are enrolled to an ongoing single-institution pilot feasibility trial.
Devimistat Plus Hydroxychloroquine Shows Early Promise in Relapsed Clear Cell SarcomaJanuary 7th 2022
No dose-limiting toxicities have been reported in the first cohort of patients with relapsed clear cell sarcoma who received the combination of devimistat and hydroxychloroquine in the dose-escalation portion of the ongoing phase 1/2 APOLLO 613 trial.
Olaparib/Temozolomide Shows Encouraging Efficacy, Manageable Safety in Uterine LeiomyosarcomaJanuary 5th 2022
In patients with advanced uterine leiomyosarcoma whose tumors harbor a BRCA-like phenotype, the combination of olaparib with temozolomide could represent a new standard of care.
Putting the Extremely Rare Cardiac Angiosarcoma in the SpotlightDecember 21st 2021
Cardiac angiosarcoma is an exceptionally rare cancer that has become national news because of the recent death of acclaimed fashion designer, Virgil Abloh. He was 41 years old when he died in November 2021, following a 2-year battle with the disease, which is an aggressive subtype of soft-tissue sarcoma.
Current Management Strategies for Desmoid Tumors Are Marked by Less Surgery, More Systemic OptionsDecember 20th 2021
Despite their rarity, the management of desmoid tumors has been an area of significant clinical change as the field has shifted away from aggressive surgical interventions to active surveillance and systemic therapies that are less morbid for patients.