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Pigmented perivascular epithelioid cell tumor (PEComa) arising from kidney: A case report
INTRODUCTION: Perivascular epithelioid cell tumor (PEComa) is a mesenchymal neoplasm composed of perivascular epithelioid cells with clear to eosinophilic cytoplasm. Pigmented PEComa arising from kidney is extraordinarily rare and sometimes can exhibit aggressive biological behavior. CASE REPORT: We...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer Health
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5591130/ https://www.ncbi.nlm.nih.gov/pubmed/27858882 http://dx.doi.org/10.1097/MD.0000000000005248 |
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author | Du, Hexi Zhou, Jun Xu, Lingfan Yang, Cheng Zhang, Li Liang, Chaozhao |
author_facet | Du, Hexi Zhou, Jun Xu, Lingfan Yang, Cheng Zhang, Li Liang, Chaozhao |
author_sort | Du, Hexi |
collection | PubMed |
description | INTRODUCTION: Perivascular epithelioid cell tumor (PEComa) is a mesenchymal neoplasm composed of perivascular epithelioid cells with clear to eosinophilic cytoplasm. Pigmented PEComa arising from kidney is extraordinarily rare and sometimes can exhibit aggressive biological behavior. CASE REPORT: We present here a rare case of pigmented renal PEComa in a 46-year-old female. The patient had complained of lumbago complicated with nausea and vomiting for 2 weeks and therefore was referred to our department. An enhanced computed scan revealed a 4 × 3 × 3 cm round-like mass in the lower pole of right kidney with inhomogeneous enhancement. The tumor cells immunestained was positive for HMB-45, focally positive for c-Kit (CD117), and negative for vimentin, S-100, AE1/AE3, CK-7, CK-18, CD-10, RCC antigen, CgA, DOG-1, EMA, smooth muscle actin, and synaptophysin. We successfully performed 3-dimensional laparoscopic resection of the neoplasm, which was then diagnosed as pigmented PEComa by postoperative pathology. No further growing lesion or metastasis was observed during a 1-year follow-up. CONCLUSION: This case report shows that pigmented renal PEComa is often presented as a renal mass with nonspecific symptoms and imaging features. The gold diagnosis of renal pigmented PEComa is mainly based on the combination of histopathology and immunohistochemistry. Complete resection by 3-dimensional laparoscopic nephron-sparing surgery can be an effective therapeutic management. |
format | Online Article Text |
id | pubmed-5591130 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Wolters Kluwer Health |
record_format | MEDLINE/PubMed |
spelling | pubmed-55911302017-09-15 Pigmented perivascular epithelioid cell tumor (PEComa) arising from kidney: A case report Du, Hexi Zhou, Jun Xu, Lingfan Yang, Cheng Zhang, Li Liang, Chaozhao Medicine (Baltimore) 7300 INTRODUCTION: Perivascular epithelioid cell tumor (PEComa) is a mesenchymal neoplasm composed of perivascular epithelioid cells with clear to eosinophilic cytoplasm. Pigmented PEComa arising from kidney is extraordinarily rare and sometimes can exhibit aggressive biological behavior. CASE REPORT: We present here a rare case of pigmented renal PEComa in a 46-year-old female. The patient had complained of lumbago complicated with nausea and vomiting for 2 weeks and therefore was referred to our department. An enhanced computed scan revealed a 4 × 3 × 3 cm round-like mass in the lower pole of right kidney with inhomogeneous enhancement. The tumor cells immunestained was positive for HMB-45, focally positive for c-Kit (CD117), and negative for vimentin, S-100, AE1/AE3, CK-7, CK-18, CD-10, RCC antigen, CgA, DOG-1, EMA, smooth muscle actin, and synaptophysin. We successfully performed 3-dimensional laparoscopic resection of the neoplasm, which was then diagnosed as pigmented PEComa by postoperative pathology. No further growing lesion or metastasis was observed during a 1-year follow-up. CONCLUSION: This case report shows that pigmented renal PEComa is often presented as a renal mass with nonspecific symptoms and imaging features. The gold diagnosis of renal pigmented PEComa is mainly based on the combination of histopathology and immunohistochemistry. Complete resection by 3-dimensional laparoscopic nephron-sparing surgery can be an effective therapeutic management. Wolters Kluwer Health 2016-11-04 /pmc/articles/PMC5591130/ /pubmed/27858882 http://dx.doi.org/10.1097/MD.0000000000005248 Text en Copyright © 2016 the Author(s). Published by Wolters Kluwer Health, Inc. All rights reserved. http://creativecommons.org/licenses/by/4.0 This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0 |
spellingShingle | 7300 Du, Hexi Zhou, Jun Xu, Lingfan Yang, Cheng Zhang, Li Liang, Chaozhao Pigmented perivascular epithelioid cell tumor (PEComa) arising from kidney: A case report |
title | Pigmented perivascular epithelioid cell tumor (PEComa) arising from kidney: A case report |
title_full | Pigmented perivascular epithelioid cell tumor (PEComa) arising from kidney: A case report |
title_fullStr | Pigmented perivascular epithelioid cell tumor (PEComa) arising from kidney: A case report |
title_full_unstemmed | Pigmented perivascular epithelioid cell tumor (PEComa) arising from kidney: A case report |
title_short | Pigmented perivascular epithelioid cell tumor (PEComa) arising from kidney: A case report |
title_sort | pigmented perivascular epithelioid cell tumor (pecoma) arising from kidney: a case report |
topic | 7300 |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5591130/ https://www.ncbi.nlm.nih.gov/pubmed/27858882 http://dx.doi.org/10.1097/MD.0000000000005248 |
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