ArticleCancer research communications2023
Systemic Treatments and Molecular Biomarkers for Perivascular Epithelioid Cell Tumors: A Single-institution Retrospective Analysis.
Article in Cancer research communications, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
16 citing papers in PubMed, 16 citations in OpenAlex.
- Multicenter phase II trial of ipilimumab and nivolumab in metastatic or unresectable perivascular epithelioid cell tumor (PEComa): a substudy ofJournal for immunotherapy of cancer · 2025Trial
- Diagnostic utility of novel marker TRIM63 ISH in the workup of uterine perivascular epithelioid cell tumors.Virchows Archiv : an international journal of pathology · 2026Article
- Article
- Durable Disease Control With Nivolumab in Malignant Epithelioid Angiomyolipoma: A Case Report.IJU case reports · 2026Article
- Review
- Case Report: Acquired CDKN2A/B deletion and mTOR inhibitor resistance in TSC2-mutant metastatic epithelioid angiomyolipoma.Frontiers in medicine · 2026Article
- A perivascular epithelioid cell neoplasm of the bladder diagnosed after six years: A case report.Urology case reports · 2025Article
- Perivascular epithelioid cell tumors of the urinary bladder: a multi-institutional clinicopathologic and molecular analysis of 21 cases.Virchows Archiv : an international journal of pathology · 2025Article
- SFPQ-TFE3 reciprocally regulates mTORC1 and induces lineage plasticity in a mouse model of renal tumorigenesis.Nature communications · 2025Article
- Cracking the code: the emerging role of immune checkpoint inhibitors in PEComas.Immunotherapy · 2025Article
- TSC2-mutated perivascular epithelioid cell tumor with partial response to mTOR inhibition: a case report and literature review.Discover oncology · 2025Article
- Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis.Frontiers in oncology · 2025Article
- Multifocal malignant perivascular epithelioid cell tumor (PEComa) of the peritoneum: A case report.International journal of surgery case reports · 2024Article
- Metastatic perirectal PEComa treated by checkpoint inhibitor immunotherapy and multimodal treatment: case report and review of the literature.Therapeutic advances in medical oncology · 2024Article
- Case report: Malignant epithelioid angiosarcoma in a Chinese female patient.Frontiers in oncology · 2024Article
- Renal Epithelioid Angiomyolipomas: Clinicopathological Features, Diagnosis, and Management.Cancer diagnosis & prognosisReview
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Perivascular epithelioid cell tumors (PEComa) are a large family of mesenchymal neoplasms, with variable clinical course. Evidence regarding treatment of advanced PEComas is scarce, with only one FDA-approved treatment available. The goals of this study were to provide data regarding systemic treatments for advanced PEComas and to identify biomarkers of prognostic relevance. This is a single-institution retrospective study of patients with advanced PEComas requiring systemic treatment, including malignant PEComa, angiomyolipoma (including the epithelioid variant), and lymphangioleiomyomatosis. Outcomes measured were overall survival (OS), first-line and combined progression-free survival (PFS), and tumor response. Kaplan-Meier, univariable, and multivariable Cox proportional hazards analysis were performed. A total of 29 patients were included, most with malignant PEComa ( Significance: This study examines systemic treatments for advanced PEComas, a rare group of sarcomas, and identifies molecular biomarkers of prognosis. Our results show that mTOR inhibitors have similar efficacy as chemotherapy, and that TFE3 overexpression, on IHC or FISH, correlates with a more aggressive disease course.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.