Sarcomas

Christian Frederick Meyer, MD, PhD, MS, discusses standard treatment options in cardiac angiosarcoma.

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.

Dr Meyer discusses the rarity of cardiac angiosarcoma, optimizing detection strategies, surgical interventions, and chemotherapy for this patient population, and why immunotherapy has the potential to transform care for patients.

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.

R. Lor Randall, MD, FACS, discusses methods of preventing early failures with compressive osseointegrative endoprosthetic devices of the femur in bone sarcomas.

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.

The FDA has granted a rare pediatric disease designation to IMX-110 for the treatment of rhabdomyosarcoma.

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.

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.

Elise Nassif, MD, discusses the evolving standard of care for patients with soft tissue sarcoma and the benefits of early phase inclusion, as well as histotype testing for this population.

R. Lor Randall, MD, FACS, elaborates on the results of a study and provided insight into future research directions in Ewing sarcoma

R. Lor Randall, MD, FACS, discusses the evolution of limb salvage technologies in bone sarcomas.

Margaret von Mehren, MD, discusses the value of ribociclib plus everolimus for patients with dedifferentiated liposarcoma, and how further research will be focused on biomarker analysis.

The targeted small molecule inhibitor of the E26 transformation-specific family of oncoproteins, ONCT-216, produced notable response and disease control rates when delivered at the recommended phase 2 dose to heavily pretreated patients with Ewing sarcoma.

The FDA has approved sirolimus albumin-bound particles for injectable suspension for the treatment of adult patients with locally advanced unresectable or metastatic malignant perivascular epithelioid cell tumor.

Breelyn Wilky, MD, discusses the diagnosis and management of desmoid tumors.

Sylvie Bonvalot, MD, PhD, HDR, discusses the long-term safety results from the phase 2/3 Act.In.Sarc trial with NBTXR3 and radiotherapy in patients with soft tissue sarcoma.

R. Lor Randall, MD, FACS, the 10-year results of a retrospective analysis, potential strategies to mitigate early failures in recipients of compressive osseointegrative endoprosthetic devices, and the need for communication between medical oncologists and orthopedic surgeons to ensure patients on chemotherapy receive optimal care.

Gina Z. D’Amato, MD, discusses optimizing treatment for patients with desmoid tumors.

Richard F. Riedel, MD, discusses the challenges of diagnosing and treating patients with desmoid tumors.

John R. Zalcberg, MBBS, PhD, discusses the INVICTUS trial examining ripretinib in patients with heavily pretreated gastrointestinal stromal tumors, the impact of the agent on the paradigm, and ongoing efforts to provide additional options to this population.

Alice Indini, MD, discusses the ongoing PULSAR trial examining pembrolizumab plus lenvatinib as a novel therapeutic option for patients with classic Kaposi sarcoma.

Ripretinib did not significantly improve progression-free survival over sunitinib in patients with gastrointestinal stromal tumor who previously received imatinib, missing the primary end point of the phase 3 INTRIGUE trial.

There is significant clinical, pathological, and outcome heterogeneity in myoepithelial tumors and prognoses for these tumors cannot be determined by a single pathological feature, but rather, several patient and tumor characteristics.

R. Lor Randall, MD, FACS, discusses initial considerations when treating patients with desmoid tumors.