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Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature
BACKGROUND: The perivascular epithelioid cell tumour (PEComa) family of tumours mainly includes renal and hepatic angiomyolipomas, pulmonary lymphangioleiomyomatosis and clear cell “sugar” tumour of the lung. Several uncommon tumours with similar morphological and immunophenotypical characteristics...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Baishideng Publishing Group Inc
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6854398/ https://www.ncbi.nlm.nih.gov/pubmed/31750334 http://dx.doi.org/10.12998/wjcc.v7.i21.3524 |
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author | Touloumis, Zisis Giannakou, Niki Sioros, Christos Trigka, Argiro Cheilakea, Maria Dimitriou, Nikoletta Griniatsos, John |
author_facet | Touloumis, Zisis Giannakou, Niki Sioros, Christos Trigka, Argiro Cheilakea, Maria Dimitriou, Nikoletta Griniatsos, John |
author_sort | Touloumis, Zisis |
collection | PubMed |
description | BACKGROUND: The perivascular epithelioid cell tumour (PEComa) family of tumours mainly includes renal and hepatic angiomyolipomas, pulmonary lymphangioleiomyomatosis and clear cell “sugar” tumour of the lung. Several uncommon tumours with similar morphological and immunophenotypical characteristics arising at a variety of sites (abdominal cavity, digestive tract, retroperitoneum, skin, soft tissue and bones) are also included in the PEComa family and are referred to as PEComas not otherwise specified. CASE SUMMARY: We present a 37-year-old female patient who underwent resection of an 8.5 cm × 8 cm × 4 cm retroperitoneal tumour, which eventually was diagnosed as PEComa of uncertain biological behaviour. Three years after the operation, the patient remains without any evidence of recurrence. A search was performed in the Medline and EMBASE databases for articles published between 1996 and 2018, and we identified 31 articles related to retroperitoneal and perinephric PEComas. We focused on sex, age, maximum dimension, histological and immunohistochemical characteristics of the tumour, follow-up and long-term outcome. Thirty-four retroperitoneal (including the present one) and ten perinephric PEComas were identified, carrying a malignant potential rate of 44% and 60%, respectively. Nearly half of the potentially malignant PEComas presented with or developed metastases during the course of the disease. CONCLUSION: Retroperitoneal PEComas are not as indolent as they are supposed to be. Radical surgical resection constitutes the treatment of choice for localized disease, while mammalian target of the rapamycin (mTOR) inhibitors constitute the most promising therapy for disseminated disease. The role of mTOR inhibitors as adjuvant or neoadjuvant therapies needs to be evaluated in the future. |
format | Online Article Text |
id | pubmed-6854398 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Baishideng Publishing Group Inc |
record_format | MEDLINE/PubMed |
spelling | pubmed-68543982019-11-20 Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature Touloumis, Zisis Giannakou, Niki Sioros, Christos Trigka, Argiro Cheilakea, Maria Dimitriou, Nikoletta Griniatsos, John World J Clin Cases Case Report BACKGROUND: The perivascular epithelioid cell tumour (PEComa) family of tumours mainly includes renal and hepatic angiomyolipomas, pulmonary lymphangioleiomyomatosis and clear cell “sugar” tumour of the lung. Several uncommon tumours with similar morphological and immunophenotypical characteristics arising at a variety of sites (abdominal cavity, digestive tract, retroperitoneum, skin, soft tissue and bones) are also included in the PEComa family and are referred to as PEComas not otherwise specified. CASE SUMMARY: We present a 37-year-old female patient who underwent resection of an 8.5 cm × 8 cm × 4 cm retroperitoneal tumour, which eventually was diagnosed as PEComa of uncertain biological behaviour. Three years after the operation, the patient remains without any evidence of recurrence. A search was performed in the Medline and EMBASE databases for articles published between 1996 and 2018, and we identified 31 articles related to retroperitoneal and perinephric PEComas. We focused on sex, age, maximum dimension, histological and immunohistochemical characteristics of the tumour, follow-up and long-term outcome. Thirty-four retroperitoneal (including the present one) and ten perinephric PEComas were identified, carrying a malignant potential rate of 44% and 60%, respectively. Nearly half of the potentially malignant PEComas presented with or developed metastases during the course of the disease. CONCLUSION: Retroperitoneal PEComas are not as indolent as they are supposed to be. Radical surgical resection constitutes the treatment of choice for localized disease, while mammalian target of the rapamycin (mTOR) inhibitors constitute the most promising therapy for disseminated disease. The role of mTOR inhibitors as adjuvant or neoadjuvant therapies needs to be evaluated in the future. Baishideng Publishing Group Inc 2019-11-06 2019-11-06 /pmc/articles/PMC6854398/ /pubmed/31750334 http://dx.doi.org/10.12998/wjcc.v7.i21.3524 Text en ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved. http://creativecommons.org/licenses/by-nc/4.0/ This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. |
spellingShingle | Case Report Touloumis, Zisis Giannakou, Niki Sioros, Christos Trigka, Argiro Cheilakea, Maria Dimitriou, Nikoletta Griniatsos, John Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature |
title | Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature |
title_full | Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature |
title_fullStr | Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature |
title_full_unstemmed | Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature |
title_short | Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature |
title_sort | retroperitoneal perivascular epithelioid cell tumours: a case report and review of literature |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6854398/ https://www.ncbi.nlm.nih.gov/pubmed/31750334 http://dx.doi.org/10.12998/wjcc.v7.i21.3524 |
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