ArticleJournal of medical case reports2021
Sustained response to pembrolizumab in recurrent perivascular epithelioid cell tumor with elevated expression of programmed death ligand: a case report.
Article in Journal of medical case reports, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 10 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
- PEComas: current concepts in diagnosis, molecular pathways, and emerging treatments.The oncologist · 2026Review
- Durable Disease Control With Nivolumab in Malignant Epithelioid Angiomyolipoma: A Case Report.IJU case reports · 2026Article
- Case Report: Acquired CDKN2A/B deletion and mTOR inhibitor resistance in TSC2-mutant metastatic epithelioid angiomyolipoma.Frontiers in medicine · 2026Article
- Cracking the code: the emerging role of immune checkpoint inhibitors in PEComas.Immunotherapy · 2025Article
- Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis.Frontiers in oncology · 2025Article
- Metastatic perirectal PEComa treated by checkpoint inhibitor immunotherapy and multimodal treatment: case report and review of the literature.Therapeutic advances in medical oncology · 2024Article
- Systemic Treatments and Molecular Biomarkers for Perivascular Epithelioid Cell Tumors: A Single-institution Retrospective Analysis.Cancer research communications · 2023Article
- Management Strategies for Adults with Locally Advanced, Unresectable or Metastatic Malignant Perivascular Epithelioid Cell Tumor (PEComa): Challenges and Solutions.Cancer management and research · 2023Review
- Dermatologic Disease-Directed Targeted Therapy (DDermatology and therapy · 2022Review
- Spectrum of Presentations and Management Strategies in Renal Angiomyolipoma.Journal of kidney cancer and VHL · 2022Article
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPerivascular epithelioid cell tumors are defined by the World Health Organization as "a collection of rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells." Whereas localized perivascular epithelioid cell tumor is typically benign and treated successfully with surgical resection, prognosis for patients with advanced or metastatic perivascular epithelioid cell tumor is unfavorable, and there is no standard curative treatment. CASE PRESENTATION: We report a Caucasian case of metastatic perivascular epithelioid cell tumor previously treated with chemotherapy and surgery with elevated surface expression of programmed cell death ligand 1. Based on this result, treatment via immune checkpoint inhibition with the monoclonal antibody pembrolizumab was pursued. After 21 cycles, the patient sustained a complete response. Therapy was stopped after the 40th cycle, and she was moved to surveillance. She remained disease free 19 months off treatment.
conclusionsThis case report of a patient with perivascular epithelioid cell tumor treated successfully with programmed cell death protein-1 targeted therapy suggests that programmed cell death ligand-1 levels should be measured in patients with perivascular epithelioid cell tumor and immunotherapy considered for recurrent or metastatic patients. Future phase II/III studies in this disease should focus on sequencing of surgery and immunotherapy with a design of curative intent.
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