Sarcomas

Michael J. Wagner, MD, discusses the challenge of diagnosing angiosarcoma.

Michael J. Wagner, MD, discusses the importance of cooperative research groups in angiocarcinoma.

Cancer experts at the Sylvester Comprehensive Cancer Center: Annual Oncology Update shared important progress across a wide range of cancers — prostate cancer, breast cancer, colon cancer, and more — that was presented at the American Society of Clinical Oncology 2022 Annual Meeting, the largest gathering of cancer clinicians and scientists in the United States.

Although systemic treatments are utilized for angiosarcoma, an aggressive and rare subset of a rare cancer, the efficacy achieved with these options is not as good as it needs to be. Immunotherapy may be able to address the unmet need for a systemic treatment in this disease.

R. Lor Randall, MD, FACS, shares the successes achieved so far with machine learning in histopathology, explained the positive effect this technology can have on institutions, and advocated for further use of technology to aid treatment advances.

Interim findings from part A of the phase 2/3 RINGSIDE trial indicated that the selective oral gamma-secretase inhibitor, AL102, was found to have favorable tolerability and early antitumor activity in patients with desmoid tumors.

R. Lor Randall, MD, FACS, discusses the similar benefits offered by radiation and surgery, improved surgical techniques, concerns with morbidity with each approach, and the next steps surgeons and radiation therapists can take to increase patient survival in nonmetastatic pelvic and sacral Ewing carcinoma.

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 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.

Treatment with larotrectinib elicited robust and durable responses, had a favorable safety profile, and sustained survival benefit in patients with central nervous system TRK fusion cancers.

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

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.

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.

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.

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

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.

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.

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.

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.

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.

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.

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.

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.

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.