Sarcoma

Following recurrence and metastasis, the patient was treated with imatinib mesylate and radiotherapy, resulting in a sustained major partial response. This report presents a rare case of aggressive sacroiliac spindle cell sarcoma with COL1A1::PDGFB fusion, expands the clinicopathologic spectrum of DFSP and its variants, and underscores the importance of molecular studies for definitive diagnosis, as well as the potential for targeted therapies in managing complex sarcoma cases.

Farnesyltransferase (FTase) inhibitors (FTIs), such as tipifarnib, inhibit HRAS membrane localization and blunt RAS effector signaling, leading to an antitumor effect in HRAS-mutant FN-RMS preclinical models...Co-targeting FTase and MEK restrained tumor progression and induced terminal myogenic differentiation. These findings highlight an effective combinatorial strategy and support its preclinical translation for patients with HRAS-mutant RMS.

In contrast, the activation or expression of the two other IFN transcription factors, the NF-κB subunit RelA and the AP-1 subunit ATF2, correlated with IFN-β induction. Our results suggest that during viral infection, activation of IRF3 does not automatically result in IFN responses at the level of individual cells, but that other factors, such as NF-κB and AP-1, are limiting for type I IFN induction.

Additional investigation like co-segregation analysis assessed the existence of this mutation in some of affected and unaffected family members and showed that probably this family is a case of familial Li-Fraumeni syndrome. This finding suggested that germline mutation of TP53 gene play an important role in initiating and spreading sarcoma in this family with Li-Fraumeni syndrome.

Herein, we present a case of intracranial CIC::DUX4 rearrangement diagnosed using DNA methylation classifiers and multiple omics diagnosis techniques. We also document the disease progression after chemoradiation therapy and subsequent in situ recurrence.

It highlights the opportunities for future research to optimize delivery systems, elucidate mechanisms of action, and establish clinical applicability. By harnessing the biological benefits of natural agents and the precision of nanomedicine, these approaches hold significant promise for improving therapeutic outcomes and reducing adverse effects in osteosarcoma treatment.

P1/2, N=47, Active, not recruiting, Centre Leon Berard | Recruiting --> Active, not recruiting | Trial completion date: Jul 2026 --> Feb 2027 | Trial primary completion date: Jan 2026 --> Feb 2027

FDCS has a unique histological morphology and immunophenotype for FDCS. It is rare for FDCS to occur in the lungs. However, owing to the lack of experience among doctors and the limitations of small biopsy specimens, FDCS can easily be misdiagnosed as lung cancer. When tumour cells exhibit the above histological features, additional immunostaining for FDC markers should be performed. The Clinical Trial Number is ChiCTR2400090730 and Reg Date is October 12, 2024.

P=N/A, N=100, Not yet recruiting, Centre Leon Berard

Targeted tyrosine kinase inhibitors (TKIs) have transformed GIST management, with imatinib as the foundational first-line therapy and subsequent agents, sunitinib, regorafenib, avapritinib, and ripretinib, addressing primary or secondary resistance driven by diverse mutational patterns. Emerging therapeutic directions include next-generation kinase inhibitors, heat shock protein inhibitors, immunotherapy, metabolic and epigenetic targeting, and biomarker-driven individualized treatment strategies. This review synthesizes contemporary advances in the histopathological, molecular, and therapeutic landscape of GISTs, emphasizing an integrated diagnostic approach and highlighting ongoing efforts to overcome therapeutic resistance and optimize personalized care.

P3, N=164, Active, not recruiting, National Cancer Institute (NCI) | Trial completion date: Dec 2025 --> Feb 2027

P1/2, N=55, Recruiting, Lantheus Medical Imaging | Not yet recruiting --> Recruiting