ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2021
Intimal sarcomas and undifferentiated cardiac sarcomas carry mutually exclusive MDM2, MDM4, and CDK6 amplifications and share a common DNA methylation signature.
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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Who cites it
28 citing papers in PubMed, 40 citations in OpenAlex.
- A First-in-Human Phase I Study of Milademetan, an MDM2 Inhibitor, in Patients With Advanced Liposarcoma, Solid Tumors, or Lymphomas.Journal of clinical oncology : official journal of the American Society of Clinical Oncology · 2023Trial
- Endovascular Diagnosis of Pulmonary Artery Intimal Sarcoma Using Intracardiac Echocardiography-Guided Biopsy.JACC. Case reports · 2026Article
- An indolent course of undifferentiated pleomorphic cardiac sarcoma mimicking myxoma: A case report.Radiology case reports · 2026Article
- Essential role of multimodality imaging in the diagnosis of a rare high-grade primary cardiac intimal sarcoma.European heart journal. Case reports · 2026Article
- Right atrial intimal sarcoma with liver metastasis: A case report and literature review.Cancer pathogenesis and therapy · 2026Article
- Intimal sarcomas (ISarc) of the cardiac chambers (CC) of the heart and great vessels (GV): a comprehensive genomic profiling (CGP) study.Cardio-oncology (London, England) · 2026Article
- Dual Left Atrial Masses Causing Inflow Obstruction: A Rare Presentation of Primary Cardiac Intimal Sarcoma.Vascular health and risk management · 2026Article
- Colonic pleomorphic undifferentiated sarcoma with a leukemoid reaction: case report.Frontiers in medicine · 2026Article
- Surgery-enabled precision oncology in an MSI-High pulmonary artery sarcoma with Lynch syndrome: a case report.Frontiers in oncology · 2026Article
- Emergency Surgery for Left Atrial Angiosarcoma Presenting with Acute Respiratory Failure: A Fatal Case Report.Surgical case reports · 2026Article
- Integrative genomic and immune landscape analysis of intimal sarcomas for emerging therapeutic targets and immunotherapy strategies.Frontiers in immunology · 2026Article
- Aortic intimal sarcoma mimicking a mycotic pararenal abdominal aortic aneurysm.Journal of vascular surgery cases and innovative techniques · 2025Article
- A Tale of Two Zebras: Cardiac Intimal Sarcoma Discovered in a Male Patient With SCAD and ADPKD.JACC. Case reports · 2025Article
- Primary cardiac sarcoma: insights from two decades of multimodal management at LMU Munich.Cardio-oncology (London, England) · 2025Article
- Article
- Genomic profiling of intimal sarcoma reveals molecular subtypes with distinct tumor microenvironments and therapeutic implications.ESMO open · 2025Article
- Pulmonary Artery Intimal Sarcoma Mimicking Intraluminal Seeding: A Case Report.Surgical case reports · 2025Article
- Primary Cardiac Intimal Sarcoma: Multi-Layered Strategy and Core Role ofDiagnostics (Basel, Switzerland) · 2024Review
- Thrombus or tumor? A case report of a rare sarcoma entity: intimal sarcoma of the pulmonary arteries.Molecular biology reports · 2024Article
- Clinical activity of pembrolizumab in refractoryTherapeutic advances in medical oncology · 2024Article
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Authors and funding
19 authors at 9 institutions in 4 countries.
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Abstract
Undifferentiated mesenchymal tumors arising from the inner lining (intima) of large arteries are classified as intimal sarcomas (ISA) with MDM2 amplification as their molecular hallmark. Interestingly, undifferentiated pleomorphic sarcomas (UPS) of the heart have recently been suggested to represent the cardiac analog of ISA due to morphological overlap and high prevalence of MDM2 amplifications in both neoplasms. However, little is known about ISAs and cardiac UPS without MDM2 amplifications and molecular data supporting their common classification is sparse. Here, we report a series of 35 cases comprising 25 ISAs of the pulmonary artery, one ISA of the renal artery and 9 UPS of the left atrium. Tumors were analyzed utilizing the Illumina Infinium MethylationEPIC BeadChip array, enabling copy number profile generation and unsupervised DNA methylation analysis. DNA methylation patterns were investigated using t-distributed stochastic neighbor embedding (t-SNE) analysis. Histologically, all ISAs and UPS of the left atrium resembled extra-cardiac UPS. All cases exhibited highly complex karyotypes with overlapping patterns between ISA and UPS. 29/35 cases showed mutually exclusive amplifications in the cell-cycle associated oncogenes MDM2 (25/35), MDM4 (2/35), and CDK6 (2/35). We further observed recurrent co-amplifications in PDGFRA (21/35), CDK4 (15/35), TERT (11/35), HDAC9 (9/35), and CCND1 (4/35). Sporadic co-amplifications occurred in MYC, MYCN, and MET (each 1/35). The tumor suppressor CDKN2A/B was frequently deleted (10/35). Interestingly, DNA methylation profiling (t-SNE) revealed an overlap of ISA and cardiac UPS. This "ISA" methylation signature was distinct from potential histologic and molecular mimics. In conclusion, our data reveal MDM4 and CDK6 amplifications in ISAs and UPS of the left atrium, lacking MDM2 amplification. We further report novel co-amplifications of various oncogenes, which may have therapeutic implications. Finally, the genetic and epigenetic concordance of ISAs and UPS of the left atrium further supports a shared pathogenesis and common classification.
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