Soft Tissue Sarcoma

Notably, within the genitourinary tract, available data suggest a relative predilection for renal involvement. This tumor confirms the broad anatomic spectrum of SEF and underscores the essential role of MUC4 immunohistochemistry and molecular testing in the evaluation of renal tumors with epithelioid cytology and prominent stromal sclerosis.

The diagnosis was confirmed by immunohistochemistry and fluorescence in situ hybridization (FISH) for PDGFB rearrangement. This report highlights the diagnostic challenges in this rare entity and emphasizes the importance of molecular confirmation.

We analyze recent advances and ongoing investigations, as well as future directions in the development of epigenetic therapies in solid tumors. Overall, epigenetic inhibitors are poised to become an expanding pillar of precision oncology, bridging chromatin biology with clinical benefit.

Consequently, molecular testing revealed mismatch repair deficiency (dMMR) among two cancers, thereby confirming the diagnosis of LS. This case suggests that UPS may represent a rare extracolonic manifestation of LS and highlights the importance of considering MMR/MSI testing in sarcomas of uncertain origin, which may have implications for diagnosis and personalized treatment strategies, including the use of immune checkpoint inhibitors.

At 3-year follow-up, the patient remains free of recurrence or metastasis. This case demonstrates the potential for EMC to mimic myoepithelial tumors and supports the use of molecular analysis when histologic or immunohistochemical findings are insufficient for diagnosis.

Genetic or pharmacological inhibition of IRE1α or spliced XBP1 (sXBP1) suppresses cell proliferation, promotes terminal myogenic differentiation, and enhances vincristine-induced cytotoxicity in RMS cells...Consistently, inducible knockdown of sXBP1 or pharmacological inhibition of IRE1α endonuclease activity significantly attenuates xenograft RMS growth. Collectively, these findings identify the IRE1α-XBP1 axis as a critical regulator of RMS growth, differentiation, and chemosensitivity, and support its therapeutic targeting in RMS.

In this study, we evaluated a novel liquid biopsy platform based on circulating cell-free DNA active chromatin (cfDNAac) profiling to identify baseline biomarkers associated with clinical benefit rate (CBR) in 30 LMS patients treated with durvalumab plus olaparib or cediranib. In this analysis, cfDNAac profiling represents a promising non-invasive strategy to predict clinical benefit from CPI-based therapy in LMS. Prospective validation in independent cohorts is warranted to clarify its clinical utility.

P=N/A, N=300, Recruiting, St. Jude Children's Research Hospital | Not yet recruiting --> Recruiting

P1, N=15, Recruiting, Tcelltech Inc. | Not yet recruiting --> Recruiting

Treatment with combined BRAF and MEK inhibition (dabrafenib and trametinib) resulted in a clinical response. In addition to this index case, a literature review identified multiple additional SS harboring BRAF V600E, supporting its role as a recurrent, potentially targetable alteration in a small subset of SS. These findings highlight the value of comprehensive genomic profiling in SS, particularly in advanced or refractory cases, to identify rare but actionable molecular events that may expand therapeutic options.

Interestingly, another component of the BAF complex, SS18, has also been reported to be fused to VEZF1 in uterine sarcoma. We propose that fusion of BAF complex components to VEZF1 leads to aberrant recruitment of chromatin-remodeling activity to VEZF1 target loci, resulting in altered chromatin architecture, dysregulated VEZF1-dependent transcription, and tumorigenesis.

Genetic or pharmacological inhibition of IRE1α or spliced XBP1 (sXBP1) suppresses cell proliferation, promotes terminal myogenic differentiation, and enhances vincristine-induced cytotoxicity in RMS cells...Consistently, inducible knockdown of sXBP1 or pharmacological inhibition of IRE1α endonuclease activity significantly attenuates xenograft RMS growth. Collectively, these findings identify the IRE1α-XBP1 axis as a critical regulator of RMS growth, differentiation, and chemoresistance, and support its therapeutic targeting in RMS.