SARCOMA

Pomalidomide is now FDA approved for treatment of Kaposi sarcoma under multiple indications.

Combination therapy strategies involving immune checkpoint inhibitors and a secondary agent have shown promise across sarcoma subtypes, according to analysis of clinical trial data that were presented at the European Society for Medical Oncology Sarcoma & GIST Symposium 2020, held in Milan, Italy.

Cabozantinib demonstrated antitumor activity as treatment of patients with advanced Ewing sarcoma and osteosarcoma, warranting further investigation for a potential new therapeutic option for this patient population, according to findings from phase II CABONE study.

Jonathan C. Trent, MD, PhD, discusses some of the recent immunotherapeutic advances in the treatment landscape for patients with sarcoma, particularly with immune checkpoint inhibitors.

Treatment with immune checkpoint inhibitors appears to elicit limited clinical activity in patients with osteosarcoma. In a study, investigators at MD Anderson Cancer Center found that certain factors like poor infiltration of the tumor by immune cells, low activity from available T cells, a lack of immune-stimulating neoantigens, and multiple immune-suppressing pathways may interfere with response to immunotherapy in these patients, according to a press release from the organization.<br /> &nbsp;

In January 2020, the FDA approved new treatment options in gastrointestinal stromal tumors, bladder cancer, and epithelioid sarcoma. The FDA also granted several Priority Review Designations, orphan drug designations, and a Fast Track designation, as well as a Breakthrough Therapy designation.

The combination of talimogene laherparepvec plus pembrolizumab led to a promising objective response rate in patients with advanced sarcoma who typically have limited treatment options, according to a phase II clinical trial&nbsp;&nbsp;published in&nbsp;<em>JAMA Oncology</em>.

In an interview with&nbsp;Targeted Oncology, Shoba A. Navai, MD,&nbsp;discussed the evolving role of CAR T-cell therapy in solid tumors, including sarcomas and how the efficacy and safety compare with CAR T cells in this space compared with hematologic malignancies.

Accelerated approval has been granted by the FDA to the methyltransferase inhibitor tazemetostat for the treatment of metastatic or locally advanced epithelioid sarcoma in adult and adolescent patients who are not eligible for complete resection.<br /> &nbsp;

SP-2577, a potent reversible LSD1 inhibitor, has been granted Fast Track Designation by the FDA for the treatment of relapsed/refractory patients with Ewing sarcoma, Salarius Pharmaceuticals, Inc. developers of the drug, announced in a press release.

The FDA has granted a Regenerative Medicine Advanced Therapy designation to ADP-A2M4 for the treatment of patients with synovial sarcoma, according to a press release from Adaptimmune Therapeutics, developers of the agent.

A New Drug Application has been accepted and granted priority review by the FDA to the investigational agent selumetinib for the treatment of patients &ge;3 years old with neurofibromatosis type 1 and symptomatic, inoperable plexiform neurofibromas, according to a press release from AstraZeneca and Merck.

The FDA plans to split up the submitted New Drug Application for avapritinib into 2 separate NDAs in the treatment of 2 different populations of patients with gastrointestinal stromal tumors, according to a press release from Blueprint Medicines.

The FDA has granted cobimetinib, a MEK inhibitor, with a breakthrough therapy designation for the treatment of adult patients with histiocytic neoplasms who do not harbor a <em>BRAF</em> V600 mutation.

The FDA has granted nirogacestat, an investigational gamma-secretase inhibitor, with a breakthrough therapy designation for the treatment of adult patients with progressive, unresectable, recurrent or refractory desmoid tumors or deep fibromatosis.

Ripretinib demonstrated a significantly improved progression-free survival compared with placebo in patients with gastrointestinal stromal tumors being treated in the fourth-line setting or beyond, according to topline results from the phase III INVICTUS trial. An NDA for ripretinib is planned for the first quarter of 2020.&nbsp;

The FDA has granted a priority review to a New Drug Application for avapritinib as a treatment for adult patients with <em>PDGFRA</em> exon 18&ndash;mutant gastrointestinal stromal tumors, regardless of prior therapy, and in the fourth-line setting for GIST.

A new drug application for tazemetostat, an EZH2 inhibitor,&nbsp;has been granted a priority review by the FDA for the treatment of patients with metastatic or locally advanced epithelioid sarcoma not eligible for curative surgery&nbsp;based on data from the&nbsp;epithelioid sarcoma cohort of a phase II trial.

Based on the ongoing and positive data observed with VEGFR targeted therapies, novel tyrosine kinase inhibitors and those already approved for other disease indications may become available to patients with bone and soft tissue sarcomas.

A new drug application for&nbsp;tazemetostat was submitted to the FDA for the treatment of patients with&nbsp;epithelioid sarcoma who are not eligible for curative surgery, according to Epizyme, the&nbsp;manufacturer of the EZH2 inhibitor.

A scheduled meeting of the FDA&#39;s&nbsp;Oncologic Drugs Advisory Committee supported the indication of pexidartinib as a treatment for adult patients with&nbsp;symptomatic tenosynovial giant cell tumor. The panel voted 12 to 3 in favor of the CSF1R inhibitor.&nbsp;

Pomalidomide was granted a breakthrough designation by the FDA for use as treatment for both patients with HIV-positive Kaposi sarcoma who have had prior chemotherapy and patients with HIV-negative Kaposi sarcoma.

The PDGFR&alpha; antagonist olaratumab (Lartruvo) will be withdrawn from the market for the treatment of patients with advanced soft tissue sarcoma due to disappointing findings in the phase III ANNOUNCE trial, according to Eli Lilly and Company.

The FDA has granted a breakthrough therapy designation to the investigational agent selumetinib for the treatment of pediatric patients &ge;3 years old with symptomatic and/or progressive, inoperable neurofibromatosis type 1

In phase I findings presented during the 2019 AACR Annual Meeting, HER2-directed CAR T-cell therapy and lymphodepletion chemotherapy was found to be safe and effective as a treatment for&nbsp;pediatric and adult patients with advanced HER2-positive sarcoma.