Sarcomas | Specialty

The OncLive Sarcomas/TGCT condition center page is a comprehensive resource for clinical news and expert insights on tenosynovial giant cell tumor (TGCT), soft tissue sarcoma, gastrointestinal stromal tumor, Ewing sarcoma, and more. This page features news articles, interviews in written and video format, and podcasts that focus on unmet needs, treatment advances, and ongoing research in sarcomas and TGCT.

Larotrectinib Elicits Durable Responses, Extends Survival in TRK+ Sarcomas

January 11th 2021

January 11, 2021 - Larotrectinib was found to induce a high response rate with long durability and improve survival in patients with TRK-positive sarcomas, according to results from an analysis of 3 clinical trials.

Dr. Dhir on Modifying Treatment for Adults Versus Pediatric Patients With Epithelioid Sarcoma

January 6th 2021

Aditi Dhir, MD, discusses modifying treatment for adults versus pediatric patients with epithelioid sarcoma.

TP53, NF1 Among Genetic Variants Identified as Precursors to Pediatric Rhabdomyosarcoma

January 6th 2021

Germline predisposition variants associated with cancer susceptibility syndromes can underlie the genetic risk for rhabdomyosarcoma, indicating that germline testing should be performed in accordance with RMS subtypes and irrespective of age.

Dr. Trent on the Role of Adjuvant Imatinib in GIST

January 6th 2021

Jonathan C. Trent, MD, PhD, discusses the role of adjuvant imatinib in gastrointestinal stromal tumors.

Ripretinib Under Investigation for Second-Line GIST in Phase 3 INTRIGUE Trial

January 5th 2021

January 5th, 2021 - Ripretinib is under investigation as a second-line treatment in patients with gastrointestinal stromal tumor versus sunitinib in the phase 3 INTRIGUE trial, which recently completed its target enrollment.

Spotlighting Management Strategies Across Sarcoma Subtypes

January 4th 2021

Faculty of a recent Institutional Perspectives in Cancer webinar on sarcoma, hosted by Sylvester Comprehensive Cancer Center, share updates in the management of gastrointestinal stromal tumor, epithelioid and synovial sarcoma, and tenosynovial giant cell tumor.

Phase 2/3 RINGSIDE Trial of AL102 Can Proceed in Desmoid Tumors

January 4th 2021

January 4, 2021 — The pivotal phase 2/3 RINGSIDE trial, which is evaluating the potent, selective oral gamma secretase inhibitor AL102, has been permitted to proceed and can potentially be used as a registrational study for adolescent and adult patients with desmoid tumors.

FDA Grants Annamycin Orphan Drug Status for Soft Tissue Sarcomas

December 29th 2020

December 29, 2020 - The FDA has granted an orphan drug designation to Annamycin for the treatment of patients with soft tissue sarcomas.

Entrectinib Continues to Elicit Durable Responses in NTRK Fusion–Positive Sarcoma

December 23rd 2020

December 23, 2020 - Entrectinib continued to demonstrate clinically meaningful, durable responses with a manageable toxicity profile in patients with NTRK fusion–positive sarcoma.

Up-Front Osteosarcoma Resection: An Approach Worth Exploring?

December 16th 2020

R. Lor Randall, MD, FACS, discusses the concept and prospect of up-front resection in osteosarcoma from the Osteosarcoma Collaborative.

Janku Highlights PFS Benefit With Dose-Escalated Ripretinib in Advanced GIST

December 15th 2020

Filip Janku, MD, PhD highlighted a dose-escalated approach of ripretinib and how it yielded a progression-free survival benefit in patients with gastrointestinal stromal tumor across all lines of treatment in a phase 1 study.

Tumor Growth Rate Associated With Outcomes in Sarcoma Treated With Immunotherapy or Targeted Therapy

December 14th 2020

December 14, 2020 — A link between tumor growth rate and clinical outcomes was established in patients with sarcoma who received treatment with immunotherapy or targeted therapy.

Ripretinib Given as Intra-Patient Dose Escalation Shows Clinically Meaningful PFS in GIST After Disease Progression

November 30th 2020

November 30, 2020 - Dose escalation with ripretinib following disease progression on the 150-mg daily dose led to a clinically meaningful benefit in patients with advanced gastrointestinal stromal tumor.

FDA Approves Sonalleve MR-HIFU System for Osteoid Osteoma in the Extremities

November 30th 2020

November 30, 2020 - The FDA has approved the Sonalleve MR-guided high-intensity focused ultrasound (MR-HIFU) system for the treatment of patients with osteoid osteoma in the extremities.

Selinexor Improves PFS in Advanced Unresectable Dedifferentiated Liposarcoma

November 25th 2020

Selinexor demonstrated a statistically significant improvement in progression-free survival compared with matching placebo in patients with advanced unresectable dedifferentiated liposarcoma, following at least 2 prior treatments.

3D Printing Leads to Improved Precision and Reconstruction in Limb Salvage Surgery in Bone Tumors, Soft Tissue Sarcomas

November 24th 2020

R. Lor Randall, MD, FACS, discusses limb salvage surgeries that have been made possible through 3D printing, and the technological advances that are helping to increase precision and turnaround times.

Devimistat Granted Orphan Drug Status for Soft Tissue Sarcoma

November 24th 2020

November 24, 2020 — The FDA has granted an orphan drug designation to devimistat for the treatment of patients with soft tissue sarcoma.

Pexidartinib Demonstrates Long-Term Efficacy, Safety in Tenosynovial Giant Cell Tumors

November 24th 2020

November 24, 2020 - Pexidartinib was found to result in prolonged efficacy with favorable safety in patients with tenosynovial giant cell tumors, according to long-term data from a pooled analysis of 3 cohorts of patients who received the agent.

Ripretinib Shows Antitumor Activity in Fourth-Line KIT/PDGFRA-Mutated Advanced GIST

November 23rd 2020

November 23, 2020 - Ripretinib demonstrated clinically meaningful activity in patients with fourth-line or later advanced gastrointestinal stromal tumor and several, heterogeneous genetic subsets of KIT/PDGFRA mutations.

Dr. Trent on the Potential Role of Immunotherapy in GIST

November 19th 2020

Jonathan C. Trent, MD, PhD, discusses the potential role of immunotherapy in gastrointestinal stromal tumors.