Management Strategies for Adults with Locally Advanced, Unresectable or Metastatic Malignant Perivascular Epithelioid Cell Tumor (PEComa): Challenges and Solutions
Anna M Czarnecka,1 Jacek Skoczylas,1 Ewa Bartnik,2,3 Tomasz Świtaj,1 Piotr Rutkowski1 1Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland; 2Institute of Genetics and Biotechnology, Faculty of Biology, Univers...
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| Format: | Article |
| Language: | English |
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Dove Medical Press
2023-07-01
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| Series: | Cancer Management and Research |
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| Online Access: | https://www.dovepress.com/management-strategies-for-adults-with-locally-advanced-unresectable-or-peer-reviewed-fulltext-article-CMAR |
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| author | Czarnecka AM Skoczylas J Bartnik E Świtaj T Rutkowski P |
| author_facet | Czarnecka AM Skoczylas J Bartnik E Świtaj T Rutkowski P |
| author_sort | Czarnecka AM |
| collection | DOAJ |
| description | Anna M Czarnecka,1 Jacek Skoczylas,1 Ewa Bartnik,2,3 Tomasz Świtaj,1 Piotr Rutkowski1 1Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland; 2Institute of Genetics and Biotechnology, Faculty of Biology, University of Warsaw, Warsaw, Poland; 3Institute of Biochemistry and Biophysics, Polish Academy of Sciences, Warsaw, PolandCorrespondence: Anna M Czarnecka, Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, W.K. Roentgena 5 street, Warsaw, 02-781, Poland, Tel + 48 22 546 2194, Email anna.czarnecka@gmail.comAbstract: PEComa (PEC tumor; perivascular epithelioid cell tumors) is a rare group of tumors of mesenchymal origin composed of perivascular epithelioid cells (PEC) with features of melanotic and smooth muscle differentiation. In this article, we would like to present the current treatment options for this group of tumors. PEComas are classified as tumors of uncertain malignant potential because recurrences occur after radical treatment. The primary treatment is surgical resection with negative margins. Due to the different locations of the tumors, often the cooperation of multispecialty surgeons is required during the operations. In locally advanced cases, cytoreduction and HIPEC may be effective but still are an experimental treatment. For nonresectable PEComa chemotherapy, mTOR inhibitors and VEGFR inhibitors are used.Keywords: PEComa, sarcoma, surgery, chemotherapy, oncology |
| first_indexed | 2024-03-13T00:56:51Z |
| format | Article |
| id | doaj.art-2e9ccae2c54b45f6824cd63f631c1c25 |
| institution | Directory Open Access Journal |
| issn | 1179-1322 |
| language | English |
| last_indexed | 2024-03-13T00:56:51Z |
| publishDate | 2023-07-01 |
| publisher | Dove Medical Press |
| record_format | Article |
| series | Cancer Management and Research |
| spelling | doaj.art-2e9ccae2c54b45f6824cd63f631c1c252023-07-06T19:09:57ZengDove Medical PressCancer Management and Research1179-13222023-07-01Volume 1561562384979Management Strategies for Adults with Locally Advanced, Unresectable or Metastatic Malignant Perivascular Epithelioid Cell Tumor (PEComa): Challenges and SolutionsCzarnecka AMSkoczylas JBartnik EŚwitaj TRutkowski PAnna M Czarnecka,1 Jacek Skoczylas,1 Ewa Bartnik,2,3 Tomasz Świtaj,1 Piotr Rutkowski1 1Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland; 2Institute of Genetics and Biotechnology, Faculty of Biology, University of Warsaw, Warsaw, Poland; 3Institute of Biochemistry and Biophysics, Polish Academy of Sciences, Warsaw, PolandCorrespondence: Anna M Czarnecka, Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, W.K. Roentgena 5 street, Warsaw, 02-781, Poland, Tel + 48 22 546 2194, Email anna.czarnecka@gmail.comAbstract: PEComa (PEC tumor; perivascular epithelioid cell tumors) is a rare group of tumors of mesenchymal origin composed of perivascular epithelioid cells (PEC) with features of melanotic and smooth muscle differentiation. In this article, we would like to present the current treatment options for this group of tumors. PEComas are classified as tumors of uncertain malignant potential because recurrences occur after radical treatment. The primary treatment is surgical resection with negative margins. Due to the different locations of the tumors, often the cooperation of multispecialty surgeons is required during the operations. In locally advanced cases, cytoreduction and HIPEC may be effective but still are an experimental treatment. For nonresectable PEComa chemotherapy, mTOR inhibitors and VEGFR inhibitors are used.Keywords: PEComa, sarcoma, surgery, chemotherapy, oncologyhttps://www.dovepress.com/management-strategies-for-adults-with-locally-advanced-unresectable-or-peer-reviewed-fulltext-article-CMARpecomasarcomasurgerychemotherapyoncology |
| spellingShingle | Czarnecka AM Skoczylas J Bartnik E Świtaj T Rutkowski P Management Strategies for Adults with Locally Advanced, Unresectable or Metastatic Malignant Perivascular Epithelioid Cell Tumor (PEComa): Challenges and Solutions Cancer Management and Research pecoma sarcoma surgery chemotherapy oncology |
| title | Management Strategies for Adults with Locally Advanced, Unresectable or Metastatic Malignant Perivascular Epithelioid Cell Tumor (PEComa): Challenges and Solutions |
| title_full | Management Strategies for Adults with Locally Advanced, Unresectable or Metastatic Malignant Perivascular Epithelioid Cell Tumor (PEComa): Challenges and Solutions |
| title_fullStr | Management Strategies for Adults with Locally Advanced, Unresectable or Metastatic Malignant Perivascular Epithelioid Cell Tumor (PEComa): Challenges and Solutions |
| title_full_unstemmed | Management Strategies for Adults with Locally Advanced, Unresectable or Metastatic Malignant Perivascular Epithelioid Cell Tumor (PEComa): Challenges and Solutions |
| title_short | Management Strategies for Adults with Locally Advanced, Unresectable or Metastatic Malignant Perivascular Epithelioid Cell Tumor (PEComa): Challenges and Solutions |
| title_sort | management strategies for adults with locally advanced unresectable or metastatic malignant perivascular epithelioid cell tumor pecoma challenges and solutions |
| topic | pecoma sarcoma surgery chemotherapy oncology |
| url | https://www.dovepress.com/management-strategies-for-adults-with-locally-advanced-unresectable-or-peer-reviewed-fulltext-article-CMAR |
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