Liposarcoma

FISH detection of NR4A3 gene rearrangements provides a crucial value for the diagnosis. It needs to be differentiated from myoepithelial tumors, chordomas and myxoid liposarcomas.

P1, N=30, Recruiting, Memorial Sloan Kettering Cancer Center | N=20 --> 30

The strong and diffuse staining pattern of desmin and SMA can be very helpful in distinguishing leiomyosarcoma from its spindle cell mimics. A novel finding is that SMA positivity was identified in malignant endothelium. Immunolabelling for CD34 and KIT provides reliable markers for vascular neoplasms and GISTs. Vimentin does not allow to distinguish between different mesenchymal malignancies in guinea pigs. Occasional positivity for keratins and S100 points to potential pitfalls and emphasised the need for a panel of immunohistochemical stains in the investigation of mesenchymal tumours.

P=N/A, N=100, Recruiting, Australia and New Zealand Sarcoma Association

Correlation of morphology with an appropriate immunophenotype and clinical-radiologic context is essential to avoid overtreatment. Complete surgical excision is the mainstay of therapy; long-term surveillance is advisable given the deep location and size.

P2, N=110, Recruiting, Oslo University Hospital | Not yet recruiting --> Recruiting

P2, N=40, Recruiting, Oslo University Hospital | Not yet recruiting --> Recruiting

Tumor-specific metastatic programs across mesenchymal malignancies (osteosarcoma, chondrosarcoma, and liposarcoma) and selected high-burden cancers (melanoma, hepatocellular carcinoma, glioblastoma, and breast cancer) are highlighted, emphasizing shared principles and divergent organotropisms. Emerging therapeutic strategies that target both the "seed" and the "soil" are also discussed, including immunotherapy combinations, stromal/ECM normalization, chemokine-axis inhibition, epigenetic reprogramming, and liquid-biopsy-enabled minimal residual disease monitoring, to prevent reactivation and improve durable control of metastatic disease.

Two patients developed local recurrences and are alive with disease, while the remaining three had no evidence of disease. We describe an unclassified fibromyxoid mesenchymal neoplasm of uncertain malignant potential characterized by recurrent biallelic NF1 LOF and widespread genomic LOH.

We separately described outcomes and treatment patterns of patients with resectable and unresectable RPS. Our findings suggest that eribulin should be considered a treatment option for unresectable RPS in clinical practice.

P2, N=40, Active, not recruiting, National Cancer Institute (NCI) | Trial completion date: May 2026 --> Dec 2026 | Trial primary completion date: May 2026 --> Dec 2026

In summary, we identified lineage-specific defects in DDLPS, with PPARG2 as the molecular mediator of differentiation state in LPS. More broadly, our findings demonstrate how defining lineage-specific mechanisms of tumor state can inform the development of nonchemotherapeutic treatment approaches.