Sarcomas

Vimseltinib was well tolerated and demonstrated promising preliminary efficacy in patients with tenosynovial giant cell tumors whose tumors are not amendable to surgical resection, according to results from a phase 1/2 study.

Elise Nassif, MD, discusses the efficacy of early-phase clinical trials in soft tissue sarcoma.

Soft tissue sarcoma was the primary cause of death in patients over the age of 75 years, indicating that age alone should not be a prohibitive factor for treatment in older patients, according to findings from a retrospective analysis that was presented at the 2021 ESMO Congress.

Brian A. Van Tine, MD, PhD, discusses the study design, key findings, adverse events, and research beyond the APROMISS study.

Alice Indini, MD, discusses the rationale for the phase 2 PULSAR trial in classic Kaposi sarcoma.

Albiruni Ryan Abdul Razak, discusses the rationale for examining durvalumab with either olaparib or cediranib in patients with LMS, key data reported with this approach, and next steps for the phase 2 DAPPER study.

R. Lor Randall, MD, discusses survey results in detail and what the sarcoma community can do to overcome clinical trial enrollment barriers for adolescent and young adult patients.

As part of a global consensus meeting, experts from several disciplines convened to define evidence-based guidelines for the treatment of patients with primary and metastatic epithelioid hemangioendothelioma with locoregional and systemic therapies, as well as palliative care.

Albiruni Ryan Abdul Razak, MRCP, MBBCh, discusses initial findings from a phase 2 trial evaluating the utility of durvalumab in combination with olaparib or cediranib in leiomyosarcoma.

The American Society for Radiation Oncology released a new clinical guideline, which was also published in Practical Radiation Oncology, outlined recommendations and patient selection for optimal radiation dosing, sequencing, and treatment planning for patients with soft tissue sarcoma.

Amputations were found to be associated with Hispanic ethnicity and male sex in patients with neoplasms of the foot.

Brian A. Van Tine, MD, PhD, discusses outcomes with catequentinib in the treatment of patients with synovial sarcoma.

Avapritinib did not improve progression-free survival vs regorafenib in patients with unresectable or metastatic gastrointestinal stromal tumors, but the agent continues to be the most active available option for a subset of patients with PDGFRA D842V–mutant disease.

The FDA has granted an orphan drug designation to sotigalimab as a potential therapeutic option for patients with soft tissue sarcoma.

Chordoma is described as an extremely rare sarcoma that attacks the bones of the spine and base of the skull.

Brian A. Van Tine, MD, PhD, discusses the rationale to evaluate catequentinib in synovial sarcoma.

The FDA has granted priority review to a new drug application for the nanoparticle albumin-bound mTOR inhibitor nab-sirolimus for the treatment of patients with malignant perivascular epithelioid cell neoplasm.

A multimodality approach to treatment, which included sequential chemotherapy and radiation therapy followed by surgery, resulted in successful resection for most patients with marginally inoperable soft tissue sarcomas.

R. Lor Randall, MD, FACS, discusses the advantages of rotationplasty for oncologic and non-oncologic indications in children and young adults.

The safety and efficacy of the oral MDM2 inhibitor milademetan is under investigation vs trabectedin, a current standard of care, in the treatment of patients with dedifferentiated liposarcoma as part of the recently initiated phase 3 MANTRA trial.

Treatment patterns differed for patients with metastatic undifferentiated pleomorphic sarcoma based on disease characteristics, age, race, combined household income, and treatment facility.

Delays between biopsy and the start of induction chemotherapy, induction chemotherapy and surgery, and surgery and consolidation chemotherapy were associated with a poor prognosis in patients with localized Ewing sarcoma.

Richard Gorlick, MD, expands on the process of identifying these novel targets in osteosarcoma and the clinical implications of this research for these patients.

Avapritinib demonstrated encouraging clinical activity in Chinese patients with PDGFRA D842V–mutant gastrointestinal stromal in a bridging study of the NAVIGATOR trial.

Sarcomas, all together, have an incidence of less than 5/100,000 persons per year, according to the Surveillance, Epidemiology, and End Results Program. Compare that with breast cancer, which impacts 129.1/100,000 women per year, or lung cancer, which has an incidence of 53.1/100,000 persons per year