SARCOMA

Avapritinib showed substantial clinical activity in patients with gastrointestinal stromal tumors with <em>KIT</em> and <em>PDGFRA</em> mutations, according to findings from the phase I NAVIGATOR trial presented at the 2018 CTOS Annual Meeting. To date, patients with GIST who harbor these mutations have typically been resistant to all available therapies.

Half of the patients with inflammatory myofibroblastic tumor (IMFT) demonstrated a response to crizotinib (Xalkori), according to results from the EORTC phase II &quot;CREATE&quot; study 90101 reported at the 2018 CTOS Annual Meeting.

The investigational agent avapritinib demonstrated encouraging response rates in patients with advanced gastrointestinal stromal tumors and PDGFR&alpha; D842V-driven GIST, according to findings presented during the 2018 Annual Meeting of the Connective Tissue Oncology Society in Rome, Italy.

During the 2018 Annual Meeting of the Connective Tissue Oncology Society, Anette Duensing, MD, assistant professor of pathology at University of Pittsburgh, discusses the need for further investigation into how treatment type impacts perceived cognitive function in patients with gastrointestinal stromal tumor.

During the 2018 Annual Meeting of the Connective Tissue Oncology Society,&nbsp;Jason Roszik, PhD, MBA, of the MD Anderson Cancer Center, discusses the use of next-generation sequencing in intimal sarcoma.<br /> &nbsp;

Patrick Sch&ouml;ffski, MD, MPH,&nbsp;discussed the key findings from the CREATE trial, the next steps for research into inflammatory myofibroblastic tumor, and the regulatory challenges for approval of drugs for rare cancers.

Practice-changing improvements in disease-free survival and overall survival rates were seen for patients with pediatric&nbsp;rhabdomyosarcoma with the addition of a course of&nbsp;low-dose maintenance chemotherapy administered after standard-of-care intensive chemotherapy.

A new drug application for larotrectinib has been granted a priority review by the FDA for&nbsp;the treatment of adult and pediatric patients with locally advanced or metastatic solid tumors with an <em>NTRK</em> gene fusion, according to Bayer and Loxo Oncology, the codevelopers of the pan-TRK inhibitor.&nbsp;

When findings of larotrectinib,&nbsp;a pan-TRK&nbsp;inhibitor co-developed by Bayer and Loxo Oncology, that generated significant excitement for the treatment of both adult and pediatric patients with TRK fusion cancers,&nbsp;were presented during the 2017 ASCO Annual Meeting, the small molecule was lauded as a future standard of care for patients with advanced solid tumors harboring a TRK fusion.

In a precision medicine success story, crizotinib (Xalkori) demonstrated a 50% objective response rate and a 100% disease control rate in patients with&nbsp;<em>ALK</em>-positive advanced, inoperable inflammatory tumors, in findings from the CREATE study presented at the 2018 AACR Annual Meeting.&nbsp;

According to findings from a phase I/II study published in&nbsp;<em>The Lancet Oncology</em>, there was an&nbsp;&ldquo;unprecedented&rdquo; objective response rate of 93% with larotrectinib&nbsp;in patients with&nbsp;TRK&nbsp;fusion&ndash;positive solid tumors.

In an interview with <em>Targeted Oncology</em>, Pollack, assistant member, Clinical Research Division, assistant professor, Division of Oncology, University of Washington, and attending physician at Seattle Cancer Care Alliance, discussed some of the advancements his lab is making in the field of sarcoma.

The new drug application for larotrectinib (LOXO-101) for treatment of patients with locally advanced or metastatic solid tumors that harbor an NTRK gene fusion&nbsp;has been completed.&nbsp;

In the sarcoma field, the potential benefit of immunotherapy is currently challenged not only by the heterogeneity of each patient, but also by the heterogeneity of each histologic subtype. William D. Tap, MD,&nbsp;discussed the role for immunotherapy in patients with soft tissue sarcomas and gastrointestinal stromal tumors (GISTs) during a presentation at the 2018 ESMO Sarcoma &amp; GIST Symposium.

Following in the footsteps of pazopanib and olaratumab, many targeted therapies are being studied in patients with soft tissue sarcomas to capitalize on the benefits of targeted therapies in&nbsp;sarcomas. Although chemotherapy-driven approaches remain the standard for treatment of sarcomas, many sensitivities to chemotherapies have appeared in patients with sarcoma subtypes. These patients&nbsp;may benefit from targeted therapy approaches.

Emanuela Palmerini, MD, Prometeo Laboratory: Clinical and Experimental Research Contract, Chemotherapy for Rare Musculoskeletal Tumors Department, Instituto Ortopedico Rizzoli, discusses optimal patient selection for denosumab (Xgeva) in patients with giant cell tumor of bone.

Patients with heavily pretreated gastrointestinal stromal tumors responded to treatment with nivolumab (Opdivo) alone and in combination with ipilimumab (Yervoy), according to findings of a randomized phase II study presented at the 2018 Gastrointestinal Cancers Symposium.

Mary C. Beckerle, PhD, has been awarded the Alfred G. Knudson Award in Cancer Genetics by the National Cancer Institute (NCI). She received her award at the NCI&rsquo;s Maryland headquarters before delivering a lecture on tumor biology.

A novel kinase switch control inhibitor, DCC-2618, demonstrated an encouraging disease control rate (DCR) of 76% at 12 weeks in heavily pretreated patients with gastrointestinal stromal tumors (GIST) treated with a daily &ge;100-mg dose, according to findings of a phase I first-in-human, dose-escalation trial.

Emanuela Palmerini, MD, Prometeo Laboratory: Clinical and Experimental Research Contract, Chemotherapy for Rare Musculoskeletal Tumors Department, Instituto Ortopedico Rizzoli, discusses efficacy results for denosumab (Xgeva) in patients with giant cell tumor of bone (GCTB).

According to findings from the phase I SCOUT trial, the novel pan-TRK inhibitor larotrectinib (LOXO-101)&nbsp;achieved a 93% response rate in pediatric patients with <em>TRK</em>&nbsp;fusion&ndash;positive solid tumors.

Brian Van Tine, MD,&nbsp;discusses the recent discoveries made for the treatment of soft tissue sarcoma and the ongoing trials for this patient population. &nbsp;&nbsp;

CMB305, an immunotherapy regimen that generates and expands anti&ndash;NY-ESO-1 T cells, demonstrated a favorable survival rate compared with other approved agents for recurrent soft tissue sarcoma, according to results presented at the 2017 ASCO Annual Meeting.

Confirmed responses were demonstrated in 3 patients with soft tissue sarcoma treated with entrectinib in the STARTRK-2 basket trial, according to the results of a case study presented at the 2017 Connective Tissue Oncology Society Annual Meeting that took place in Maui, Hawaii.

Brian Van Tine, MD, PhD, assistant professor, Washington University School of Medicine, discusses the development of olaratumab (Lartruvo) in soft tissue sarcoma.