PAX3 (Paired Box 3)

No specific clustering was seen among different fusion types or tumor grade. Our findings support that BSS represents a unique and molecularly distinct sinonasal sarcoma with a characteristic DNA methylation signature, independent of gene fusion partners or histologic progression, providing a valuable tool for the classification of challenging cases.

Collectively, our findings demonstrate that vestigial-like 4 is an important regulator of neurogenesis and neural crest formation. Although vestigial-like 4 can bind to tead proteins in the embryo, its function does not depend solely on this interaction, suggesting a complex level of regulation with which vestigial-like 4 regulates early steps in development and differentiation.

Their structures were elucidated using 1D and 2D NMR experiments and their absolute configurations determined using semisynthetic and electronic circular dichroism methods. The gonystylones were found to be cytotoxic to rhabdomyosarcoma cells at low micromolar concentrations (3-19 μM).

Despite overall excellent survival rates, challenges remain in reducing long-term morbidity and personalising treatment through genetic and molecular knowledge. This review offers current recommendations and highlights the importance of a multi-disciplinary approach in the diagnosis, staging and treatment of orbital RMS.

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.

Notably, we identified PAX3 as a transcriptional target associated with Nrf2 activation, suggesting a potential link between Nrf2 signaling and adipogenic regulation. Together, these findings reveal a previously underappreciated role for Nrf2 in human adipogenesis.

Eleven years after treatment, the patient remains free of recurrence. Understanding BSNS is essential to avoid excessive intervention, and confirmation of PAX3 rearrangement by FISH or equivalent molecular testing is crucial for accurate diagnosis.

Cancer chemotherapy mainly vincristine, dactinomycin, and cyclophosphamide, surgery, radiotherapy, and brachytherapy were the common keywords of treatment. The more common keywords such as age, facial neoplasms, mouth neoplasms, orbital neoplasms, gingiva, mandible, DNA-binding proteins, gene silencing, and Rh30 cell line were reported by stomatologists. This study is the first comprehensive report of the scientometric characteristics of RMS publications by pediatricians and stomatologists, highlighting the need for increased awareness among clinicians to avoid diagnostic delays and ensure timely treatment.

P1/2, N=31, Active, not recruiting, National Cancer Institute (NCI) | Trial completion date: Nov 2025 --> Mar 2026

In the myoblast model system, transduction studies with exogenous miR-17-92 or miRNA-sponge expression constructs indicated that miR-17-92 is necessary but not sufficient for oncogenic transformation. Together, these findings establish a cooperative transcriptional axis in FP-RMS involving P3F and MYCN that activates MIR17HG through a distal regulatory element, thereby contributing to oncogenic behavior and uncovering a novel mechanistic vulnerability.

This case highlights not only the biological aggressiveness of ARMS, but also the systemic delays in diagnosis that may occur in resource-limited settings. Its educational value lies in raising awareness about diagnostic inequity in pediatric oncology and emphasizing the need for early suspicion and timely referral in atypical clinical presentations.

Knocking down YOD1 or inhibiting it by G5 could suppress FP-RMS growth both in vitro and in vivo, through promoting the degradation of both PAX-FOXO1 and N-Myc. Collectively, our results identify that YOD1 promotes RMS progression by regulating the PAX3-FOXO1-N-Myc positive feedback loop, and highlight YOD1 inhibition as a promising therapeutic strategy that concurrently reduces the levels of both oncogenic proteins.