Perivascular Epithelioid Cell Tumor (PEComa) of the Sigmoid Colon: Case Report and Literature Review
Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms characterized by perivascular epithelioid cell proliferation. They can occur in various organs, but colonic PEComas are exceptionally rare, showing diagnostic challenges due to their nonspecific clinical presentation and s...
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| Format: | Article |
| Language: | English |
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MDPI AG
2025-06-01
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| Series: | Current Oncology |
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| Online Access: | https://www.mdpi.com/1718-7729/32/6/330 |
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| author | Gintare Slice Rokas Stulpinas Tomas Poskus Marius Kryzauskas |
| author_facet | Gintare Slice Rokas Stulpinas Tomas Poskus Marius Kryzauskas |
| author_sort | Gintare Slice |
| collection | DOAJ |
| description | Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms characterized by perivascular epithelioid cell proliferation. They can occur in various organs, but colonic PEComas are exceptionally rare, showing diagnostic challenges due to their nonspecific clinical presentation and similar features to those of other colorectal tumors. We present a case of a 61-year-old female with defecation accompanied by blood clots, initially diagnosed with a suspected tumor in the sigmoid colon. Despite initial biopsy yielding non-informative material, repeat colonoscopy and imaging studies revealed a malignant tumor with multinucleated giant (osteoclast-like) cells and probable p53 mutation, most likely of mesenchymal origin. Robotic surgical resection was performed, and ultimately pathological examination refined the diagnosis as a malignant PEComa of the colon. This case demonstrates the importance of considering PEComa in the differential diagnosis of colonic tumors. Further research is needed to ascertain the clinical behavior and optimal treatment for colonic PEComas. |
| format | Article |
| id | doaj-art-b9cd9005965b49028fcda4fbe45ebe5c |
| institution | Kabale University |
| issn | 1198-0052 1718-7729 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Current Oncology |
| spelling | doaj-art-b9cd9005965b49028fcda4fbe45ebe5c2025-08-20T03:27:23ZengMDPI AGCurrent Oncology1198-00521718-77292025-06-0132633010.3390/curroncol32060330Perivascular Epithelioid Cell Tumor (PEComa) of the Sigmoid Colon: Case Report and Literature ReviewGintare Slice0Rokas Stulpinas1Tomas Poskus2Marius Kryzauskas3Faculty of Medicine, Vilnius University, 03101 Vilnius, LithuaniaNational Center of Pathology, Affiliate of Vilnius University Hospital Santaros Klinikos, 08406 Vilnius, LithuaniaClinic of Gastroenterology, Nephrourology, and Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, 03101 Vilnius, LithuaniaClinic of Gastroenterology, Nephrourology, and Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, 03101 Vilnius, LithuaniaPerivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms characterized by perivascular epithelioid cell proliferation. They can occur in various organs, but colonic PEComas are exceptionally rare, showing diagnostic challenges due to their nonspecific clinical presentation and similar features to those of other colorectal tumors. We present a case of a 61-year-old female with defecation accompanied by blood clots, initially diagnosed with a suspected tumor in the sigmoid colon. Despite initial biopsy yielding non-informative material, repeat colonoscopy and imaging studies revealed a malignant tumor with multinucleated giant (osteoclast-like) cells and probable p53 mutation, most likely of mesenchymal origin. Robotic surgical resection was performed, and ultimately pathological examination refined the diagnosis as a malignant PEComa of the colon. This case demonstrates the importance of considering PEComa in the differential diagnosis of colonic tumors. Further research is needed to ascertain the clinical behavior and optimal treatment for colonic PEComas.https://www.mdpi.com/1718-7729/32/6/330perivascular epithelioid cell tumorPEComacolonrectalgastrointestinalneoplasm |
| spellingShingle | Gintare Slice Rokas Stulpinas Tomas Poskus Marius Kryzauskas Perivascular Epithelioid Cell Tumor (PEComa) of the Sigmoid Colon: Case Report and Literature Review Current Oncology perivascular epithelioid cell tumor PEComa colon rectal gastrointestinal neoplasm |
| title | Perivascular Epithelioid Cell Tumor (PEComa) of the Sigmoid Colon: Case Report and Literature Review |
| title_full | Perivascular Epithelioid Cell Tumor (PEComa) of the Sigmoid Colon: Case Report and Literature Review |
| title_fullStr | Perivascular Epithelioid Cell Tumor (PEComa) of the Sigmoid Colon: Case Report and Literature Review |
| title_full_unstemmed | Perivascular Epithelioid Cell Tumor (PEComa) of the Sigmoid Colon: Case Report and Literature Review |
| title_short | Perivascular Epithelioid Cell Tumor (PEComa) of the Sigmoid Colon: Case Report and Literature Review |
| title_sort | perivascular epithelioid cell tumor pecoma of the sigmoid colon case report and literature review |
| topic | perivascular epithelioid cell tumor PEComa colon rectal gastrointestinal neoplasm |
| url | https://www.mdpi.com/1718-7729/32/6/330 |
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