CASE REPORT article
Front. Oncol.
Sec. Radiation Oncology
Volume 15 - 2025 | doi: 10.3389/fonc.2025.1551663
This article is part of the Research TopicCase Reports in Radiation Oncology: 2025View all 13 articles
Case report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis
Provisionally accepted
- 1Department of Radiation Oncology, School of Medicine, University of Virginia, Charlottesville, United States
- 2Ventura County Medical Center (VCMC), Ventura, California, United States
- 3Department of Radiation Oncology, School of Medicine, Indiana University Bloomington, Indianapolis, Indiana, United States
- 4Southeast Health Medical Center, Dothan, Alabama, United States
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Perivascular epithelioid cell tumors (PEComas) are rare, typically benign soft tissue tumors that can develop at various anatomic sites. Malignant PEComas are rarer entities but may present aggressively with metastasis to the lungs, or local recurrence years after initial presentation. In unresectable or metastatic cases, treatment options are limited due to resistance of PEComas to chemotherapy and radiotherapy.The present case describes a 59-year-male with a highly aggressive malignant PEComa which ultimately invaded the mediastinum and replaced the right middle and lower lobes of the lung despite systemic therapy with oral sirolimus and definitive radiotherapy. As only three prior cases have described malignant PEComas invading the mediastinum, we highlight the clinical course of such an aggressive cancer and review current treatment paradigms.
Keywords: Perivascular epithelioid cell tumors, intensity-modulated radiotherapy, 3D Conformal Radiotherapy, Palliative radiotherapy, mTOR inhibitors, VEGF INHIBITORS
Received: 26 Dec 2024; Accepted: 23 Jul 2025.
Copyright: © 2025 Leach, Margam S, Foster and Adkison. 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: Daniel Friel Leach, Department of Radiation Oncology, School of Medicine, University of Virginia, Charlottesville, United States
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