Liposarcoma

Identification of PLAG1 rearrangement is crucial for confirming the diagnosis. With the growing number of molecularly defined soft-tissue tumours of diagnostic and prognostic significance, incorporating molecular analysis is essential in the evaluation of soft-tissue lesions.

P1, N=24, Active, not recruiting, Michael Wagner, MD | Trial primary completion date: Jun 2026 --> Jan 2027

Distinct from previously reported conventional angiomatoid fibrous histiocytoma cases with low proliferative activity and negative liposarcoma-related markers, the present case is characterized by atypical dual positive expression of CDK4/MDM2 and a markedly elevated Ki-67 proliferation index, which fills the clinical data gap of atypical angiomatoid fibrous histiocytoma with liposarcoma-mimicking immunophenotype. This study further clarifies the differential diagnostic criteria between angiomatoid fibrous histiocytoma and dedifferentiated liposarcoma, verifies that isolated CDK4/MDM2 immunohistochemical positivity cannot independently support the diagnosis of liposarcoma, and highlights the irreplaceable value of molecular testing in the definitive diagnosis of atypical angiomatoid fibrous histiocytoma, providing a reliable reference for pathologists to avoid diagnostic errors in similar rare soft tissue tumors.

The authors conclude that ecDNA amplification serves as a credible adverse prognostic indicator and holds promise for refining risk stratification and guiding treatment strategies. However, they stress that clinical adoption remains constrained by the absence of standardized, scalable, and reproducible detection.

Collectively, these findings provide a disease-oriented, cross-subtype systems-level view of the transcriptional changes associated with doxorubicin treatment in liposarcoma. This work is intended as a hypothesis-generating framework that may inform future functional studies and integrative approaches aimed at understanding therapeutic response and disease progression.

P2, N=75, Active, not recruiting, M.D. Anderson Cancer Center | Trial completion date: Jun 2026 --> Jun 2027 | Trial primary completion date: Jun 2026 --> Jun 2027

These findings highlight the clinical relevance of immune infiltration in retroperitoneal DDLPS and LMS. Moreover, they support the rationale for further exploration of the immune architecture for prognostic biomarkers and development of targeted immunotherapeutic strategies to improve the clinical outcomes of the patients.

P2/3, N=140, Completed, National Cancer Institute (NCI) | Active, not recruiting --> Completed | Trial completion date: Dec 2026 --> Mar 2026

This case highlights the diagnostic challenge of rare sarcomas presenting with serosal effusions and emphasizes the importance of integrating cytopathological, radiological, and molecular testing for accurate diagnosis and optimal management. Preventing misdiagnosis of this tumor as other histological subtypes is of great clinical significance, while IHC features and FISH can provide important clues for its accurate diagnosis.

Continued progress in biomarker-driven strategies and rational combination therapies is expected to further refine personalized treatment approaches and improve outcomes for patients with advanced liposarcoma.

In this review, we discuss the biology of MDM2 as an oncogene and summarize the current literature on MDM2 gene amplification in surgical pathology. We further outline the available diagnostic methods for assessing MDM2 status and highlight the potential diagnostic, prognostic, and therapeutic implications of this alteration.

Patients with DDLPS treated in US community settings are heterogeneous in terms of demographic and clinical characteristics, biomarker testing, treatment, and outcomes. The relatively poor survival rates suggest an unmet need for better therapeutics.