Liposarcoma

Our case study revealed that the pathogenic BRCA2 c.5946del germline variant can be associated with an unusual tumour spectrum, which may lead to a delayed diagnosis of a hereditary tumour predisposition. Thus, upfront genetic testing using large multigene panels or whole-genome sequencing in encouraged, especially in cases with a prominent family history.

ORR and PFS were highly variable across sarcoma histologic subtype. In this large analysis, KS, ASPS, AS, MFS, and UPS demonstrated the highest ORR and longest PFS while osteosarcoma, SS, and LPS had the lowest ORR and shortest PFS. PD-L1 expression was also associated with increased ORR. Our findings provide further insight into understanding the sarcoma histologic and immunologic factors that correspond with response to ICIs.

Our study reveals different lipid metabolism, several transcriptional pathways between WDLPS and DDLPS, and examines several serum markers associated with the prognosis of RLPS. These findings provide a vital basis for future endeavors in diagnosing and predicting the prognosis of retroperitoneal liposarcoma with different differentiations.

P1/2, N=110, Recruiting, Sotio Biotech Inc. | Trial completion date: Dec 2041 --> Dec 2042 | Trial primary completion date: Apr 2026 --> Oct 2027

P=N/A, N=167, Completed, Taproot Health | Recruiting --> Completed | N=100000 --> 167 | Trial completion date: Oct 2031 --> Oct 2024 | Trial primary completion date: Oct 2029 --> Oct 2024

WDLPS, however, demonstrated upregulation of HIF-1A and TGF-β when compared to DDLPS by targeted transcriptome analysis and orthogonal validation by RT-qPCR suggesting activation of EMT pathway in WDLPS when compared to DDLPS. Our findings implicate a putative role for dysregulation in glucose metabolism, TGF-β and HIF1 signaling in the pathogenesis of both WD/DDLPS suggesting a possible proinflammatory tumor environment within WDLPS and subsequent activation of the TGF-β signaling pathway.

This paper presents a case of dedifferentiated liposarcoma with osteosarcomatous differentiation in a rare location: the mediastinum. Correct diagnosis is of importance for appropriate choice of therapy. Clinicians should be aware of the presence of a dedifferentiated liposarcoma within a mass on the mediastinum and enhancing treatment and management strategies for affected patients.

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.

P3, N=108, Active, not recruiting, Sarcoma Alliance for Research through Collaboration | Recruiting --> Active, not recruiting | Trial completion date: Nov 2024 --> Dec 2026 | Trial primary completion date: May 2024 --> Dec 2025

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

These findings provide valuable insights for veterinary medicine and wildlife conservation, particularly regarding the role of environmental stressors in cancer development. This study highlights the need for comprehensive diagnostic approaches in wildlife pathology.

These findings explain the unusual mechanism underlying trabectedin's effectiveness against MLPS by pinpointing the chimera's role in inducing the differentiation block responsible for MLPS pathogenesis. Additionally, the findings hint at a potential mechanism of resistance acquired in vivo.

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

P2, N=87, Active, not recruiting, Adaptimmune

Morphological diagnosis alone can accurately diagnose lipomas. However, it has a propensity to overdiagnose ALT/WDLPS. Thus, MDM2 FISH should be used more proactively, not only for lesions with obvious morphological abnormalities, but also for lipomatous tumors that are clinically suggestive of ALT/WDLPS.

P2, N=14, Terminated, Shanghai Pharma Biotherapeutics USA Inc. | N=33 --> 14 | Trial completion date: Mar 2025 --> Oct 2024 | Recruiting --> Terminated | Trial primary completion date: Mar 2025 --> Oct 2024; Although no safety issues with SPH4336 were identified, efficacy in liposarcoma patient was less than anticipated.

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.

Laboratory measures and utilizing NGS assay when needed, could be implemented when encountering such problematic "low-level" MDM2 amplification specimens to avoid misdiagnosis and misuse of targeted therapy. Future studies are needed to better characterize and investigate such findings.

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

Infiltrating lipomas, while benign, can mimic malignant adipocytic tumors such as atypical lipomatous tumors (ALTs) and well-differentiated liposarcomas (WDLS), complicating diagnosis. This case underscores the importance of combining histopathology, immunohistochemistry (IHC), and molecular testing, particularly murine double minute clone 2 (MDM2) amplification, to distinguish between benign and malignant tumors.

MutPTEN vs wtPTEN was associated with worse OS in advanced STS. If confirmed, our findings could be helpful for prognostic stratification in clinical practice and for further understanding the molecular mechanisms of STS.

P2, N=40, Active, not recruiting, National Cancer Institute (NCI) | Trial completion date: Dec 2024 --> Dec 2025 | Trial primary completion date: Dec 2024 --> Dec 2025

To the best of our knowledge, this is the first description of a primary liposarcoma of the spleen reported in the literature. Due to the local recurrence risk of liposarcomas, even after R0 resection, we recommended long-term periodic follow-up.

The search for novel agents from existing classes (tyrosine kinase inhibitors) with efficacy in liposarcoma also continues. Combination therapies as well as biomarker identification for patient selection of therapies warrant ongoing exploration.

Final diagnosis of DDLS was rendered. The patient had no systemic lesions elsewhere on positron emission tomography computed tomography scan.

P1, N=20, Recruiting, Memorial Sloan Kettering Cancer Center | N=10 --> 20

P2, N=77, Active, not recruiting, Alliance for Clinical Trials in Oncology | Recruiting --> Active, not recruiting

Long-term follow-up is crucial for managing LPSs due to their high recurrence rate. This case highlights the effectiveness of surgical intervention in recurrent intrathoracic LPSs and underlines the need for continued research into adjuvant therapies to improve patient outcomes.

Drugs that target FUS::DDIT3 and its downstream molecules for treating MLS are widely utilized in both clinical practice and experimental studies, and some of them have demonstrated promising results. This article reviews the findings of relevant research, providing an overview of the oncogenic mechanisms of the FUS::DDIT3 fusion protein in MLS, as well as recent advancements in its therapy.

P2, N=7, Active, not recruiting, Adaptimmune | Trial completion date: Jul 2024 --> Jul 2025

We identified three unique sarcoma cell cycle phenotypes that have prognostic significance. This performs similarly to the AJCC staging system.

P1/2, N=114, Recruiting, Milton S. Hershey Medical Center | Not yet recruiting --> Recruiting

P2, N=66, Recruiting, National Cancer Institute (NCI) | Suspended --> Recruiting

The 2-gene signature model further confirmed the improved risk assessment performance of BAG1 and BAG2: high risk patients displayed poor prognosis. BAG1 and BAG2 are supposed to be potential prognostic biomarkers for LPS and have impacts on liposarcomagenesis and immune infiltration in distinctive manners, which may function as potential therapy targets (BAG1 agonists/BAG2 inhibitors) for LPS.

The radiomics model extended with automatic and minimally interactive segmentation methods accurately differentiated between lipomas and ALTs in two large, multi-center external cohorts, and in prospective validation, performing similar to expert radiologists, possibly limiting the need for invasive diagnostics. Hanarth fonds.

Using flow cytometry, we observed distinct changes in fat pad macrophage populations, with a particular increase in M2-like macrophages. Taken together, we find that overexpression of Gli2 in DDLPS cells alters their differentiation capacity and interactions between tumor cells and macrophages, highlighting a novel role for this developmental transcription factor in liposarcoma pathogenesis.

This review provides an overview of the current knowledge on the clinicoradiological features, pathogenesis, histopathology, and treatment of ASCPLT. In addition, we will discuss the differential diagnosis of this new entity.

P=N/A, N=0, Available, Adaptimmune

We report a patient with a large retroperitoneal pleomorphic liposarcoma harboring a rare mutation of the MEN1 gene not previously reported to be associated with soft tissue sarcomas. This report expands the known spectrum of MEN1-associated cancers.

This evidence suggests that disruption of the nuclear lamina and nucleoporins is crucial for ALS pathomechanisms. Combined with patient-derived hiPSC-LMNs and autopsy samples, this mouse model might provide a more reliable understanding of ALS pathogenesis and might aid in the development of therapeutic strategies.

The comprehensive treatment approach for MLS demonstrates superior local recurrence rates and survival rates compared to most soft tissue sarcomas. Current research focuses on developing effective therapies for unresectable or advanced disease based on genomic and phenotypic characteristics as well as the immune-tumor microenvironment.

This case report highlights the use of tumor resection via the cervical approach as a good option for esophageal liposarcoma.