Sarcomas/TGCT | Specialty

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.

Nirogacestat Significantly Improves PFS in Progressing Desmoid Tumors

May 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.

Selinexor Improves PFS, Time to Next Treatment in Advanced, Metastatic Dedifferentiated Liposarcoma

May 18th 2022

Selinexor monotherapy prolonged progression-free survival, time to next treatment, and reduced pain in patients with advanced, refractory dedifferentiated liposarcoma.

FDA Accepts NDA Resubmission for Pedmark in Prevention of Platinum-Induced Ototoxicity in Solid Tumors

April 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.

Pathologic Fractures Linked With Poor Outcomes in Osteosarcoma, Ewing Sarcoma

April 15th 2022

For patients with osteosarcoma or Ewing sarcoma who develop pathologic fractures, the prognosis remains poor; however, there is still the possibility for limb salvage therapy.

FDA Grants Orphan Drug Designation to Veyonda for Soft Tissue Sarcoma

March 29th 2022

The FDA has granted an orphan drug designation to Veyonda, a novel proprietary formulation of idronoxil, for use as a potential therapeutic option in patients with soft tissue sarcoma.

Randall Talks Rich AAOS 2022 Program, Packed With Engaging Discussions

March 28th 2022

R. Lor Randall, MD, discusses takeaways from the American Academy of Orthopaedic Surgeons 2022 Annual Meeting.

NDA Resubmitted to FDA for Pedmark in Prevention of Platinum-Induced Ototoxicity in Solid Tumors

March 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 Option

March 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.

Histology-Specific Trials and Drug Development Strategy Pave the Path to Progress in Soft Tissue Sarcoma

March 1st 2022

William D. Tap, MD, discusses the key challenges faced in the treatment of patients with soft tissue sarcoma and efforts being made to overcome them.

NCCN Guidelines List Ripretinib as Preferred Fourth-Line Option in Gastrointestinal Stromal Tumor

February 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.

Nearly 300 Novel Agents Currently in Development for Rare Cancers

February 24th 2022

New agents for rare cancers make up approximately 35% of drugs in the pipeline for all rare diseases, according to a report from the Pharmaceutical Research and Manufacturers of America.

Investigators Explore Metformin to Prevent Cancers in Li-Fraumeni Syndrome Carriers

February 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 Cancer

February 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.

Dr. Van Tine on the Methods Utilized in the Spearhead 1 Trial in Sarcoma Subtypes

February 10th 2022

Brian A. Van Tine, MD, PhD, discusses the methods utilized in the phase 2 Spearhead 1 trial evaluating afamitresgene autoleucel in patients with advanced synovial sarcoma or myxoid/round cell liposarcoma.

Brachytherapy Represents a Potential Strategy in AYA Patients With Rhabdomyosarcoma

February 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 GIST

February 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

February 7th 2022

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.

Dr. Tap on Developing Treatment Opportunities in Soft Tissue Sarcomas

February 2nd 2022

William D. Tap, MD, discusses developing treatment opportunities in soft tissue sarcomas.

FDA Grants Rare Pediatric Disease Designation to Novel Radiopharmaceutical for Osteosarcoma

February 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 Disease

January 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.