Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis
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 unresectabl...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2025-08-01
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| Series: | Frontiers in Oncology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fonc.2025.1551663/full |
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| author | Daniel F. Leach Srivikram Margam S Marissa Foster Jarrod B. Adkison |
| author_facet | Daniel F. Leach Srivikram Margam S Marissa Foster Jarrod B. Adkison |
| author_sort | Daniel F. Leach |
| collection | DOAJ |
| description | 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 the resistance of PEComas to chemotherapy and radiotherapy. The present case describes a 59-year-old man 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. |
| format | Article |
| id | doaj-art-e8e6bce9b719483aa2ed3c6f6000f8ad |
| institution | DOAJ |
| issn | 2234-943X |
| language | English |
| publishDate | 2025-08-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Oncology |
| spelling | doaj-art-e8e6bce9b719483aa2ed3c6f6000f8ad2025-08-20T03:07:01ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2025-08-011510.3389/fonc.2025.15516631551663Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasisDaniel F. Leach0Srivikram Margam S1Marissa Foster2Jarrod B. Adkison3Department of Radiation Oncology, The Ohio State University, Columbus, OH, United StatesFamily Medicine, Ventura County Medical Center, Ventura, CA, United StatesDepartment of Radiation Oncology, Indiana University School of Medicine, Indianapolis, IN, United StatesRadiation Oncology, Southeast Health, Dothan, AL, United StatesPerivascular 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 the resistance of PEComas to chemotherapy and radiotherapy. The present case describes a 59-year-old man 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.https://www.frontiersin.org/articles/10.3389/fonc.2025.1551663/fullperivascular epithelioid cell tumorsintensity-modulated radiotherapy3D conformal radiotherapypalliative radiotherapymTOR inhibitorsVEGF inhibitors |
| spellingShingle | Daniel F. Leach Srivikram Margam S Marissa Foster Jarrod B. Adkison Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis Frontiers in Oncology perivascular epithelioid cell tumors intensity-modulated radiotherapy 3D conformal radiotherapy palliative radiotherapy mTOR inhibitors VEGF inhibitors |
| title | Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis |
| title_full | Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis |
| title_fullStr | Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis |
| title_full_unstemmed | Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis |
| title_short | Case Report: Malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis |
| title_sort | case report malignant perivascular epithelioid cell tumor with aggressive mediastinal invasion and pulmonary metastasis |
| topic | perivascular epithelioid cell tumors intensity-modulated radiotherapy 3D conformal radiotherapy palliative radiotherapy mTOR inhibitors VEGF inhibitors |
| url | https://www.frontiersin.org/articles/10.3389/fonc.2025.1551663/full |
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