ArticleCancer2020
Systemic treatments in MDM2 positive intimal sarcoma: A multicentre experience with anthracycline, gemcitabine, and pazopanib within the World Sarcoma Network.
Article in Cancer, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers, 1 of them a synthesis that pooled it.
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Who cites it
27 citing papers in PubMed, 1 synthesis or guideline pooled it, 46 citations in OpenAlex.
- A systematic review and meta-analysis of pazopanib efficacy and adverse effects in sarcomas.Journal of translational medicine · 2026Pooled it
- Pulmonary Artery Intimal Sarcoma Successfully Treated With Surgery and Adjuvant Chemotherapy: A Case Report.Clinical case reports · 2026Article
- Endovascular Diagnosis of Pulmonary Artery Intimal Sarcoma Using Intracardiac Echocardiography-Guided Biopsy.JACC. Case reports · 2026Article
- Severe mitral stenosis due to primary cardiac intimal sarcoma: case report.European heart journal. Case reports · 2026Article
- Integrative genomic and immune landscape analysis of intimal sarcomas for emerging therapeutic targets and immunotherapy strategies.Frontiers in immunology · 2026Article
- Case Report: Adjuvant radiation therapy for cardiac intimal sarcoma with long-term disease control.Frontiers in oncology · 2026Article
- Aortic intimal sarcoma mimicking a mycotic pararenal abdominal aortic aneurysm.Journal of vascular surgery cases and innovative techniques · 2025Article
- Multi-omics analysis revealed potential use of immunotherapy and CDK4/6 inhibitors in intimal sarcoma.Frontiers in immunology · 2025Article
- Genomic profiling of intimal sarcoma reveals molecular subtypes with distinct tumor microenvironments and therapeutic implications.ESMO open · 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
- The challenging approach of a young patient with a primary intimal sarcoma of the heart: a case report.European heart journal. Case reports · 2024Article
- A case of left atrial intimal sarcoma with rhabdomyosarcoma differentiation: a case report and literature review.Frontiers in oncology · 2024Article
- Case report: Navigating treatment pathways for cardiac intimal sarcoma withFrontiers in oncology · 2024Article
- Clinical activity of pembrolizumab in refractoryTherapeutic advances in medical oncology · 2024Article
- Systemic Therapy in Advanced Pleomorphic Liposarcoma: a Comprehensive Review.Current treatment options in oncology · 2023Review
- Multimodal Imaging and Management of a Metastatic Cardiac Intimal Sarcoma.CASE (Philadelphia, Pa.) · 2023Article
- Intimal Sarcoma with MDM2/CDK4 Amplification and p16 Overexpression: A Review of Histological Features in Primary Tumor and Xenograft, with Immunophenotype and Molecular Profiling.International journal of molecular sciences · 2023Review
- Neoplastic Ruptured Cerebral Aneurysm Caused by Intimal Sarcoma: A Case Report.NMC case report journal · 2022Article
- Safety and effectiveness of gemcitabine for the treatment of classic Kaposi's sarcoma without visceral involvement.Therapeutic advances in medical oncology · 2022Article
Corrections and comments
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Authors and funding
39 authors at 20 institutions in 7 countries.
Funding
Abstract
backgroundIntimal sarcoma (InS) is an exceedingly rare neoplasm with an unfavorable prognosis, for which new potentially active treatments are under development. We report on the activity of anthracycline-based regimens, gemcitabine-based regimens, and pazopanib in patients with InS.
methodsSeventeen sarcoma reference centers in Europe, the United States, and Japan contributed data to this retrospective analysis. Patients with MDM2-positive InS who were treated with anthracycline-based regimens, gemcitabine-based regimens, or pazopanib between October 2001 and January 2018 were selected. Local pathological review was performed to confirm diagnosis. Response was assessed by RECIST1.1. Recurrence-free survival (RFS), progression-free survival (PFS) and overall survival were computed by Kaplan-Meier method.
resultsSeventy-two patients were included (66 anthracycline-based regimens; 26 gemcitabine-based regimens; 12 pazopanib). In the anthracycline-based group, 24 (36%) patients were treated for localized disease, and 42 (64%) patients were treated for advanced disease. The real-world overall response rate (rwORR) was 38%. For patients with localized disease, the median RFS was 14.6 months. For patients with advanced disease, the median PFS was 7.7 months. No anthracycline-related cardiac toxicity was reported in patients with cardiac InS (n = 26). For gemcitabine and pazopanib, the rwORR was 8%, and the median PFS was 3.2 and 3.7 months, respectively.
conclusionThis retrospective series shows the activity of anthracycline-based regimens in InS. Of note, anthracyclines were used in patients with cardiac InS with no significant cardiac toxicity. The prognosis in patients with InS remains poor, and new active drugs and treatment strategies are needed.
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