ArticleTherapeutic advances in medical oncology2024
Metastatic perirectal PEComa treated by checkpoint inhibitor immunotherapy and multimodal treatment: case report and review of the literature.
Article in Therapeutic advances in medical oncology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed.
- 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
- Malignant uterine PEComa: a narrative literature review and challenging case report.Journal of cancer research and clinical oncology · 2026Review
- Cracking the code: the emerging role of immune checkpoint inhibitors in PEComas.Immunotherapy · 2025Article
- Article
- PEComa progressed by mTOR inhibitor therapy with anti-VEGFR TKI combined with PD-1 inhibitor: a case report.Frontiers in oncology · 2025Article
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Authors and funding
11 authors.
Funding
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Abstract
Malignant PEComas are an extremely rare subtype of soft tissue sarcomas. Here, we report the case of a man presenting with a perirectal PEComa and liver metastasis. Since the tumor harbored a tumor mutational burden of 23/Mb and a programmed death-ligand 1 tumor positivity score of 50%, the patient was treated with pembrolizumab as a second line of systemic therapy, in combination with everolimus. This combined therapy led to a near-complete response of the primary tumor and a partial response of the metastasis. Radioembolization of the liver metastasis was performed due to isolated liver progression, and the pelvic tumor was treated by radiotherapy because of pelvic symptoms. The disease is still stable after 13 months of pembrolizumab plus everolimus and multimodal treatment. This case shows that malignant PEComas can display molecular features associated with sensitivity to checkpoint inhibitors. The use of checkpoint inhibitors may be a relevant therapeutic strategy in these patients. It is also the first report on selective internal radiation therapy in PEComas.
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Registered trials
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