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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...

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Autores principales: Elousrouti, Layla Tahiri, Mouaddine, Amal, Fadlallah, Imane, Elhitmy, Sofia, Elloudi, Sara, Mernissi, Fatimazahra, Elidrissi, Mohammed, Hammas, Nawal, Elfatemi, Hinde, Chbani, Laila
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2023
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.
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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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