Sarcoma

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.

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.

New data show pain worsened faster, and more dramatically, in patients given placebo versus selinexor for dedifferentiated liposarcoma, as measured by the European Organisation for Research and Treatment of Cancer’s 30-item core quality of life questionnaire.

The agent annamycin, which offers the potential to fulfill unmet medical needs of traditional anthracyclines, has been granted fast track designation for the treatment of soft tissue sarcoma lung metastases.

Based on findings from a study looking at niraparib plus cytotoxic agents, investigators have commenced a combination study of the niraparib plus temozolomide and irinotecan triplet in patients with pretreated Ewing sarcoma.

Data published in Cancer the journal found that the response to neoadjuvant therapy in patients with retroperitoneal sarcoma following surgery was fair overall, with data suggesting the potential for disease progression as a predictive tool for patient survival.

A study presented at The Society for Immunotherapy of Cancer’s (SITC) 35th Anniversary Annual Meeting suggested that ipilimumab (Yervoy) plus nivolumab (Opdivo) for patients with metastatic or unresectable angiosarcoma was well-tolerated.

The phase 3 SEAL study evaluating single agent, oral selinexor (Xpovio) versus matching placebo in patients with advanced unresectable dedifferentiated liposarcoma met its primary end point of a statistically significant increase in progression-free survival.

However, though the CD56 target was confirmed to be expressed in the tumor types studied, the clinical efficacy of lorvotuzumab mertansine was limited.