Sarcoma

National Comprehensive Cancer Network guidelines will now include ripretinib as the ideal second-line treatment for patients with unresectable/metastatic gastrointestinal stromal tumors who are intolerant to sunitinib.

Nirogacestat may be a noteworthy therapeutic advance for patients with desmoid tumors, according to an expert from Memorial Sloan Kettering Cancer Center.

Findings from a retrospective cohort study suggest that circulating tumor DNA may be an important tool for risk-stratified treatment strategies in rhabdomyosarcoma.

Data from a ctDNA analysis of the phase 3 INTRIGUE study indicate that KIT mutational status may be associated with response to certain Tyrosine kinase inhibitors in GIST, according to an expert from the Yale Cancer Center in New Haven, Massachusetts.

An expert from the Smilow Cancer Hospital and Yale Cancer Center indicates that findings from the ctDNA analysis of the phase 3 INTRIGUE study were “provocative” and that the subsequent phase 3 INSIGHT study has the potential to be practice changing should data be positive.

Findings from the phase 3 INTRIGUE trial highlighted improved median progression-free survival in patients with KIT exon–mutated gastrointestinal stromal tumors compared with sunitinib.

Adults and children with unresectable or metastatic alveolar soft part sarcoma—one of the rarest types of sarcoma—can now receive treatment with atezolizumab, which was given the nod of approval by the FDA.

Results from a phase 2 trial indicated that subgroups of patients with advanced or metastatic sarcoma may benefit from durvalumab plus tremelimumab, with data encouraging further evaluation in the population.

Investigators indicated that patients with soft tissue sarcomas should be counseled on receiving moderately hypofractionated radiotherapy vs conventionally fractionated radiotherapy, especially if it results in care at a sarcoma specialty center.

Findings from a retrospective cohort study indicated that radiotherapy treatment for breast cancer appeared to be a significant risk factor in developing thoracic soft tissue sarcoma.

A trial investigating seclidemstat for Ewing and FET-rearranged sarcomas has voluntarily paused enrollment following an unexpected death.

After review by an independent data monitoring committee, the phase 2 ENVASARC trial, analyzing envafolimab monotherapy or in combination with ipilimumab for patients with undifferentiated pleomorphic sarcoma and myxofibrosarcoma, will proceed as planned.

Patients with metastatic unresectable leiomyosarcoma in the first line benefitted more from doxorubicin and trabectedin compared with doxorubicin alone.

Patients with gastrointestinal stromal tumors may benefit from 68GA-RM26 PET/CT vs 18F-FDG, according to findings presented at the 2022 Society of Nuclear Medicine & Molecular Imaging Annual Meeting.

Ifosfamide leads to slightly better event-free and overall survival vs topotecan/cyclophosphamide in relapsed or refractory Ewing sarcoma.

A 5-day prophylactic postoperative intravenous antibiotic regimen did not reduce the rate of surgical site infections vs a 1-day regimen and increased the risk of antibiotic complications for patients with lower extremity bone tumors.

Patients with pretreated gastrointestinal stromal tumors who were treated with ripretinib did not reach the primary end point of progression-free survival superiority vs sunitinib in the phase 3 INTRIGUE study.

IMX-110, which was granted a rare pediatric disease designation for patients with rhabdomyosarcoma, is currently being assessed as part of a phase 1b/2a study.

The interim results of a phase 1b/2 trial showed that patients with soft tissue sarcoma and lung metastases who were treated with annamycin experienced positive clinical activity.

The addition of temozolomide to vincristine and irinotecan appears to be a new standard of care for adult and pediatric patients with relapsed/refractory rhabdomyosarcoma, according to the European Paediatric Soft Tissue Sarcoma Group.

Pediatric patients with medulloblastoma may experience benefit from 177Lu-omburtamab-DTPA, which was granted a rare pediatric disease designation by the FDA.

Refractory gastrointestinal stromal tumors can potentially be treated by using a KIT inhibitor combination of PLX9486 and sunitinib.

The recommended phase 2 dose for lenvatinib was 14 mg/m2 in relapsed/refractory osteosarcoma.

A new set of guidelines for utilizing radiation therapy in adult patients with soft tissue sarcoma has been published by the American Society for Radiation Oncology.

Findings from the phase 2 DART trial demonstrated that the combination of ipilimumab and nivolumab may have potential for the treatment of patients with angiosarcoma.

CancerNetwork® sat down with Sylvie Bonvalot, MD, PhD, at the 2021 ASCO Annual Meeting to talk about treatment options for high-grade or grade II sarcoma.

The mTOR inhibitor nab-sirolimus may be the first agent to receive FDA approval as therapy specifically for perivascular epithelioid cell tumors.

Pimitespib significantly improved progression-free survival while prolonging overall survival compared with placebo to treat patients with advanced gastrointestinal stromal tumor.

Patients with advanced synovial sarcoma who received treatment with catequentinib experienced improved disease control and progression-free survival compared with dacarbazine.

Results of the phase 2 SPEARHEAD-1 trial were presented at the 2021 ASCO Annual Meeting, with a high overall response rate reported with afamitresgene autoleucel in advanced synovial sarcoma or myxoid/round cell liposarcoma.