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5d
ATRX loss in sarcomas is associated with dysregulated gene and transposable element expression, loss of DNA methylation, and worse survival. (PubMed, ESMO Rare Cancers)
ATRX mutational status may serve as a potential biomarker for prognosis and therapeutic stratification. Future clinical trials investigating epigenetic therapies could offer novel treatment strategies for ATRX-deficient sarcomas.
Journal
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ATRX (ATRX Chromatin Remodeler)
6d
XPO1 inhibitor KPT-330 disrupts the core transcriptional regulatory circuitry of dedifferentiated liposarcoma by modulating the translation process. (PubMed, Oncogene)
Furthermore, we identify a synergistic antitumor effect between KPT-330 and translation inhibitors, including everolimus and homoharringtonine. Notably, the disruptive impact of KPT-330 on CRC homeostasis extends to other cancer cell lineages, underscoring its broad mechanistic relevance. Collectively, our findings elucidate a novel mechanism through which KPT-330 destabilizes CRC via translational dysregulation and highlight its potential therapeutic utility in combination regimens for DDLPS.
Journal
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XPO1 (Exportin 1)
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everolimus • Xpovio (selinexor) • Synribo (omacetaxine mepesuccinate)
15d
Targeting eIF4A-dependent translation in genetically complex sarcoma. (PubMed, JCI Insight)
The eIF4A inhibitor CR-1-31B effectively suppressed tumor growth and induced apoptosis in DDLS, MFS, and UPS patient-derived cell lines and mouse xenografts...Genomic analysis of patient tumors revealed that YAP and WWTR1 were frequently amplified or gained in DDLS, MFS, and UPS and were associated with worse clinical outcomes. Together, our findings identify a new strategy for targeting the Hippo pathway in incurable forms of sarcoma based on inhibition of eIF4A-dependent translation of the key oncogenic transcription factors YAP and TAZ.
Journal
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YAP1 (Yes associated protein 1) • WWTR1 (WW Domain Containing Transcription Regulator 1)
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CR-1-31-B
15d
RNA-based next-generation sequencing strategy for screening MDM2-amplified sarcomas. (PubMed, Sci Rep)
The algorithm also performed robustly in the TCGA-SARC data, although with a slightly different optimal cutoff, likely due to differences in gene panels, depth of sequencing, gene coverage, and bioinformatics pipelines. This study presents an effective strategy for screening DDLPS and potentially other MDM2-amplified sarcomas.
Journal • Next-generation sequencing
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MDM2 (E3 ubiquitin protein ligase)
16d
STC-15-24202: STC-15 as a Part of Combination Therapy With Toripalimab in Selected Advanced Cancers and as Monotherapy in Participants With Selected Sarcomas (clinicaltrials.gov)
P1/2, N=107, Recruiting, STORM Therapeutics LTD | N=188 --> 107 | Trial completion date: Jun 2027 --> Jun 2028 | Trial primary completion date: Dec 2026 --> Dec 2027
Enrollment change • Trial completion date • Trial primary completion date
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Loqtorzi (toripalimab-tpzi) • STC-15
19d
Small Round Cell Tumors of Soft Tissue and Skeleton in Children and Youth: A Narrative and TruSight-Based Illustrative Review of Diagnostic Cases. (PubMed, Adv Anat Pathol)
Immunohistochemistry plays a crucial role in interpreting a specific diagnosis or narrowing the differential diagnosis of SRCTs. Molecular genetic investigations are essential, particularly in cases exhibiting atypical or overlapping histologic and immunohistological features.
Journal
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BCOR (BCL6 Corepressor) • EWSR1 (EWS RNA Binding Protein 1)
20d
Spatially-resolved single cell atlas of liposarcoma reveals lineage hierarchies, immune niches, and regulatory circuits. (PubMed, bioRxiv)
H3K27ac-marked enhancers were enriched near adipocytic marker genes in WDLPS and mesenchymal markers in DDLPS. Together, these findings reveal the cellular heterogeneity of tumor and immune compartments across liposarcoma subtypes and identify regulatory programs driving their differentiation states.
Journal
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TCF7L2 (Transcription Factor 7 Like 2) • PPARG (Peroxisome Proliferator Activated Receptor Gamma) • GLI2 (GLI Family Zinc Finger 2) • FABP4 (Fatty Acid Binding Protein 4) • FOSL2 (FOS Like 2)
29d
Madelung's disease involving the scrotum: a case report and diagnostic pitfalls with liposarcoma. (PubMed, Front Oncol)
The patient remained recurrence-free at 12-month follow-up. This case emphasizes the critical role of multimodal medical imaging, rigorous histopathological evaluation, and molecular testing in establishing an accurate diagnosis and guiding appropriate management, while highlighting that Madelung's disease may occasionally present with atypical imaging features, such as nodules and septa which closely mimic a malignant lesion.
Journal
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MDM2 (E3 ubiquitin protein ligase) • CDK4 (Cyclin-dependent kinase 4)
29d
Distinct immunologic patterns of response and resistance to anti-PD-1/PD-L1-based immunotherapy in patients with soft tissue sarcoma. (PubMed, Front Immunol)
Our findings highlight peripheral immune profiles as candidate biomarkers for predicting and monitoring ICI efficacy in STS. Incorporating these immune markers could refine patient selection, reduce unnecessary toxicity, and support adaptive treatment strategies for patients with this rare and heterogenous cancer.
Journal • PD(L)-1 Biomarker • IO biomarker
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CD8 (cluster of differentiation 8)
29d
Overall survival from the open-label phase II trial of palbociclib in patients with advanced well-differentiated/dedifferentiated liposarcoma. (PubMed, ESMO Open)
This study provides the longest prospective OS data for palbociclib in WD/DD LPS. CDK4/6 inhibition remains a relevant treatment option, especially for patients unsuitable for cytotoxic therapy. CDH18 expression was associated with clinical outcomes and represents an exploratory biomarker signal that warrants independent validation in future prospective, biomarker-focused studies.
P2 data • Journal
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MDM2 (E3 ubiquitin protein ligase) • CDH18 (Cadherin 18)
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Ibrance (palbociclib)
1m
A Challenging Case of Pleomorphic Lipoma of the Shoulder Mimicking Liposarcoma. (PubMed, Am J Case Rep)
It underscores the importance of thorough histopathologic evaluation and immunohistochemical profiling to establish an accurate diagnosis and avoid unnecessary aggressive treatment. Complete surgical excision remains essential for diagnosis and management.
Journal
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RB1 (RB Transcriptional Corepressor 1) • CDK4 (Cyclin-dependent kinase 4) • CD34 (CD34 molecule)
1m
Trial completion date
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CTAG1B (Cancer/testis antigen 1B)
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cyclophosphamide • fludarabine IV • letetresgene autoleucel (GSK3377794)