Soft Tissue Sarcoma

P=N/A, N=18, Not yet recruiting, Technical University of Munich

P2, N=30, Recruiting, University of Wisconsin, Madison | Suspended --> Recruiting

When administered above the microdose, ABY-029 demonstrated high correlation to EGFR expression and contrast values that were encouraging for translation to clinical practice. Contrast was similar to those observed with antibody agents, but with substantially reduced imaging-to-resection time, and no drug-related adverse events.

The expression of chemoresistance genes has been detected, including KLF4 (doxorubicin and ifosfamide), ULK1, LUM, GPNMB, and CAVIN1 (doxorubicin), and AHNAK2 (gemcitabine) in tumor cells and ETS1 (gemcitabine) in TME. This study provides the first description of the single-cell transcriptome of UPS with resistance to two lines of chemotherapy, showcasing the gene expression in subpopulations of tumor cells and TME, which may be potential markers for personalized cancer therapy.

The prognosis of these tumors is highly dependent on factors such as tumor location and stage at diagnosis, and given their aggressive nature, a multidisciplinary approach may be required that combines surgical resection, chemotherapy, and radiation therapy, among other options. In this review, we provide a synopsis of the pathophysiology of DSRCTs and the latest diagnostic advancements, including the utility of molecular profiling and novel biomarkers.

Finally, we leverage the molecular profiles to identify vulnerabilities, including sensitivity to ATR and PARP inhibition as potential treatment for feline FSA. In conclusion, this detailed landscape provides a rich resource to identify target candidates and therapeutic vulnerabilities within and across species and supports feline FSA as relevant models for the human disease.

Genomic profiling of sarcomas offers valuable insights into their molecular drivers and can support personalized therapeutic approaches. Further research is needed to validate these findings and optimize treatment strategies for sarcoma patients.

On microscopic evaluation, the tumor was composed of solid nests and sheets of small rounded cells, and on Immunohistochemical (IHC) evaluation, the tumor cells showed intense cell-membranous immunoactivity for MIC2 protein (CD99). In the differential diagnosis of any invasive pelvic tumor in young women, pPNET should be considered.

P1, N=18, Recruiting, University of Southern California | Trial completion date: Jan 2026 --> Jan 2027 | Trial primary completion date: Jan 2025 --> Jan 2026

P=N/A, N=40, Recruiting, Ruijin Hospital | Not yet recruiting --> Recruiting

P3, N=600, Recruiting, Institut Claudius Regaud | Trial completion date: Mar 2025 --> Oct 2032 | Trial primary completion date: Mar 2025 --> Oct 2032

This study of multiple cohorts identifies CHEK2 as a candidate susceptibility gene for DFSP. Additional epidemiologic and functional studies are needed to further characterize this potential gene-tumor relationship.

P1, N=18, Enrolling by invitation, The Hospital for Sick Children | Trial completion date: Dec 2024 --> Dec 2025 | Trial primary completion date: Dec 2023 --> Jun 2025

P=N/A, N=35, Completed, Medical College of Wisconsin | Active, not recruiting --> Completed

Patients with fusion-positive N1 ARMS carry a poor prognosis. Adequate local treatment of LN improved survival.

P2, N=127, Completed, Centre Oscar Lambret | Active, not recruiting --> Completed | Trial completion date: Mar 2025 --> Sep 2024

P=N/A, N=30, Not yet recruiting, University Medical Center Groningen

Myxosarcomas elicit a B-cell and Treg-rich immune response; PWTs stimulate a T-cell-rich and Treg-poor reaction. The immune system response may contribute to the more aggressive behavior of myxosarcomas and the more indolent course of PWTs.

P=N/A, N=25, Completed, Leiden University Medical Center | Recruiting --> Completed | N=120 --> 25

Our study provides valuable insights into the microenvironment and immunological characteristics of RMS, offering crucial information for further research and potential therapeutic strategies.

Compared to DFSP, AP-DFSP shows more benign clinical and histological features with a good prognosis. Surgical intervention leads to a significant reduction in tumor burden and dramatically increases the likelihood of complete remission.

We presented the third case of synchronous PTC and SFTT. The co-occurrence of these tumors is likely incidental. However, further studies are needed to assess the physiopathology and molecular alterations of this association.

P2, N=28, Recruiting, University of Colorado, Denver | Trial completion date: Nov 2025 --> Nov 2026 | Trial primary completion date: Nov 2024 --> Nov 2025

P=N/A, N=24, Active, not recruiting, Fox Chase Cancer Center | Trial completion date: Oct 2024 --> Oct 2027 | Trial primary completion date: Jun 2021 --> Oct 2026

P2, N=130, Completed, Italian Sarcoma Group | Active, not recruiting --> Completed

P2, N=57, Active, not recruiting, Dana-Farber Cancer Institute | Trial primary completion date: Aug 2025 --> Jul 2024

Herein, we report the first case of primary intraosseous SFT of the mandible with complete documentation of its characteristic immunohistochemical and molecular features. Diagnosis of such unusual presentations may be further complicated by the challenging histomorphologic diversity of SFT.

P1/2, N=6, Terminated, University of Washington | Trial completion date: Jan 2027 --> Oct 2024 | Active, not recruiting --> Terminated; Terminated due to end of funding

The article Imatinib Mesylate in Advanced Dermatofibrosarcoma Protuberans: Pooled Analysis of Two Phase II Clinical Trials, received most citations...The research on DFSP faces several clinical challenges. This study provides novel insights into future research directions and scientific decisions for DFSP.

One patient with a high tumor mutational burden (37/Mb) responded to pembrolizumab... Our findings suggest distinct molecular patterns in phyllodes tumors compared to other soft tissue sarcomas, with potential implications for treatment selection. The identification of specific genetic alterations associated with treatment resistance may guide therapeutic decision-making, while the presence of actionable mutations in select cases indicates potential opportunities for targeted therapy approaches.

P1/2, N=30, Active, not recruiting, National Taiwan University Hospital | Trial completion date: Jun 2023 --> Dec 2024

P1/2, N=358, Recruiting, GI Innovation, Inc. | N=92 --> 358 | Trial completion date: Jun 2025 --> Apr 2027 | Trial primary completion date: Apr 2025 --> Nov 2025

While each of these entities is less common than Ewing sarcoma, it is important to distinguish these tumors for correct diagnosis, prognostication, and potential treatment management. The focus of this review will cover CIC-rearranged sarcoma, BCOR-altered sarcomas, and EWSR1-non-ETS sarcomas to include recent developments in desmoplastic small round cell tumor as well as sarcomas with EWSR1/FUS::NFATc2 and EWSR1::PATZ1 gene fusions, highlighting the clinical, morphologic, and immunophenotypic clues to the diagnosis with recognition of each molecular diagnostic hallmark.

P=N/A, N=310, Active, not recruiting, McMaster University | Recruiting --> Active, not recruiting | N=200 --> 310 | Trial completion date: Dec 2030 --> Dec 2027 | Trial primary completion date: Dec 2030 --> Dec 2027

P1/2, N=34, Active, not recruiting, Eli Lilly and Company | Trial completion date: Oct 2024 --> Oct 2025

Case 1: A 44-year-old male started trabectedin as second-line therapy after initial chemotherapy, which included doxorubicin. He then began trabectedin and has maintained a response for 10 months to date. Compared to other chemotherapies, trabectedin demonstrated potentially higher efficacy and a favorable safety profile for patients with myxoid liposarcoma harboring the FUS/CHOP fusion gene.

P1, N=448, Not yet recruiting, Hansoh BioMedical R&D Company

P1, N=12, Not yet recruiting, Pure Biologics S.A.

P1/2, N=62, Active, not recruiting, Massachusetts General Hospital | Recruiting --> Active, not recruiting | Trial primary completion date: Jul 2024 --> Jul 2025

P2, N=49, Not yet recruiting, Fudan University

Of these genes, COL6A3 and BGN predicted overall survival and metastasis-free survival in independent cohorts of 46 and 38 UPS tumors, respectively. COL6A3 was most predictive of overall survival in UPS patients and outperformed an established sarcoma prognostic gene panel at predicting metastasis in UPS.

P1/2, N=0, Withdrawn, University of California, San Francisco | N=70 --> 0 | Not yet recruiting --> Withdrawn