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Primary Cutaneous Malignant Perivascular Epithelioïd Cell Tumor (PEComa): Case Report With Review of the Literature
Perivascular epithelioïd cell tumor (PEComa) is a mesenchymal neoplasm with epithelioïd or spindled morphology with numerous thin-walled capillaries between tumor cells. They co-express markers of both melanocytic and smooth muscle differentiation. PEComas are rare, presenting in numerous anatomic s...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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SAGE Publications
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10288419/ https://www.ncbi.nlm.nih.gov/pubmed/37360521 http://dx.doi.org/10.1177/2632010X231178629 |
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author | Elousrouti, Layla Tahiri Mouaddine, Amal Fadlallah, Imane Elhitmy, Sofia Elloudi, Sara Mernissi, Fatimazahra Elidrissi, Mohammed Hammas, Nawal Elfatemi, Hinde Chbani, Laila |
author_facet | Elousrouti, Layla Tahiri Mouaddine, Amal Fadlallah, Imane Elhitmy, Sofia Elloudi, Sara Mernissi, Fatimazahra Elidrissi, Mohammed Hammas, Nawal Elfatemi, Hinde Chbani, Laila |
author_sort | Elousrouti, Layla Tahiri |
collection | PubMed |
description | Perivascular epithelioïd cell tumor (PEComa) is a mesenchymal neoplasm with epithelioïd or spindled morphology with numerous thin-walled capillaries between tumor cells. They co-express markers of both melanocytic and smooth muscle differentiation. PEComas are rare, presenting in numerous anatomic sites including lung, kidney, liver, genitourinary tract, soft tissue, and skin. Primary cutaneous PEComas are very rare entity, and malignant ones are even more uncommon. Herein, we report the case of a 92-year-old female which was presenting with 7 cm exophytic, ulcerated, hemorrhagic nodular tumor, and rapidly growing for 8 months over the right thigh. On histologic examination, we found a dermal neoplasm formed by an atypical clear cell tumor with numerous branching capillaries between tumor cells. The mitotic count was found 6 mitotic figures/10 HPF. On immunohistochemistry, tumor cells co-expressed smooth muscle and melanocytic markers, CD10, and CD68. Based on these findings, the diagnosis of primary cutaneous malignant perivascular epithelioïd cell tumor (PEComa) was made. The large size (7 cm), the count of mitoses (6 mitotic figures/10 HPF), and the nuclear pleomorphism argued for malignancy. The absence of soft tissue or visceral localization argued for the cutaneous primitive origin. Adjuvant radiotherapy and targeted therapy with mTOR inhibitor (nab-sirolimus) was indicated. To the best of our knowledge, this is only the eighth case of a primary cutaneous malignant PEComa reported in the literature to date. |
format | Online Article Text |
id | pubmed-10288419 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | SAGE Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-102884192023-06-24 Primary Cutaneous Malignant Perivascular Epithelioïd Cell Tumor (PEComa): Case Report With Review of the Literature Elousrouti, Layla Tahiri Mouaddine, Amal Fadlallah, Imane Elhitmy, Sofia Elloudi, Sara Mernissi, Fatimazahra Elidrissi, Mohammed Hammas, Nawal Elfatemi, Hinde Chbani, Laila Clin Pathol Case Report Perivascular epithelioïd cell tumor (PEComa) is a mesenchymal neoplasm with epithelioïd or spindled morphology with numerous thin-walled capillaries between tumor cells. They co-express markers of both melanocytic and smooth muscle differentiation. PEComas are rare, presenting in numerous anatomic sites including lung, kidney, liver, genitourinary tract, soft tissue, and skin. Primary cutaneous PEComas are very rare entity, and malignant ones are even more uncommon. Herein, we report the case of a 92-year-old female which was presenting with 7 cm exophytic, ulcerated, hemorrhagic nodular tumor, and rapidly growing for 8 months over the right thigh. On histologic examination, we found a dermal neoplasm formed by an atypical clear cell tumor with numerous branching capillaries between tumor cells. The mitotic count was found 6 mitotic figures/10 HPF. On immunohistochemistry, tumor cells co-expressed smooth muscle and melanocytic markers, CD10, and CD68. Based on these findings, the diagnosis of primary cutaneous malignant perivascular epithelioïd cell tumor (PEComa) was made. The large size (7 cm), the count of mitoses (6 mitotic figures/10 HPF), and the nuclear pleomorphism argued for malignancy. The absence of soft tissue or visceral localization argued for the cutaneous primitive origin. Adjuvant radiotherapy and targeted therapy with mTOR inhibitor (nab-sirolimus) was indicated. To the best of our knowledge, this is only the eighth case of a primary cutaneous malignant PEComa reported in the literature to date. SAGE Publications 2023-06-21 /pmc/articles/PMC10288419/ /pubmed/37360521 http://dx.doi.org/10.1177/2632010X231178629 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage). |
spellingShingle | Case Report Elousrouti, Layla Tahiri Mouaddine, Amal Fadlallah, Imane Elhitmy, Sofia Elloudi, Sara Mernissi, Fatimazahra Elidrissi, Mohammed Hammas, Nawal Elfatemi, Hinde Chbani, Laila Primary Cutaneous Malignant Perivascular Epithelioïd Cell Tumor (PEComa): Case Report With Review of the Literature |
title | Primary Cutaneous Malignant Perivascular Epithelioïd Cell Tumor (PEComa): Case Report With Review of the Literature |
title_full | Primary Cutaneous Malignant Perivascular Epithelioïd Cell Tumor (PEComa): Case Report With Review of the Literature |
title_fullStr | Primary Cutaneous Malignant Perivascular Epithelioïd Cell Tumor (PEComa): Case Report With Review of the Literature |
title_full_unstemmed | Primary Cutaneous Malignant Perivascular Epithelioïd Cell Tumor (PEComa): Case Report With Review of the Literature |
title_short | Primary Cutaneous Malignant Perivascular Epithelioïd Cell Tumor (PEComa): Case Report With Review of the Literature |
title_sort | primary cutaneous malignant perivascular epithelioïd cell tumor (pecoma): case report with review of the literature |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10288419/ https://www.ncbi.nlm.nih.gov/pubmed/37360521 http://dx.doi.org/10.1177/2632010X231178629 |
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