CASE REPORT article
Front. Oncol.
Sec. Cancer Molecular Targets and Therapeutics
Volume 15 - 2025 | doi: 10.3389/fonc.2025.1628650
This article is part of the Research TopicBeyond Conventional Biomarkers: Unlocking Immunotherapy Response Through Novel Biomarkers or Combinatorial ApproachesView all 4 articles
PEComa Progressed by mTOR Inhibitor Therapy with Anti-VEGFR TKI Combined with PD-1 Inhibitor：a case report
Provisionally accepted
- Cancer Hospital Shenzhen Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shenzhen, China
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Malignant perivascular epithelioid cell tumors (PEComas) are rare tumors in which the PI3K/AKT/mTOR pathway is critical for pathogenesis and is often associated with mutations in the TSC1/TSC2 genes. First-line mTOR inhibitors are an effective treatment, but metastatic PEComas eventually develop drug resistance and disease progression. A 76-year-old woman presenting with a painful soft tissue mass in the buttocks was diagnosed with malignant PEComa with local invasion and multiple distant metastases, and the tumor cells harbored a TSC2 gene mutation. After disease progression by mTOR inhibitor treatment, immunotherapy combined with anti-angiogenic therapy was enabled, which enabled the patient to achieve a 3-year survival benefit and provided a reference for the treatment of patients with refractory malignant PEComa.
Keywords: malignant perivascular epithelioid cell tumor, TSC2 mutation, mTOR inhibitor, Anti-VEGFR TKI, PD-1 inhibitor
Received: 14 May 2025; Accepted: 14 Aug 2025.
Copyright: © 2025 Wang, Sun, Guan and Yang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Boyan Yang, Cancer Hospital Shenzhen Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shenzhen, China
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