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Malignant Mesenteric Perivascular Epithelioid Cell Neoplasm Presenting as an Intra-Abdominal Fistula in a 49-Year-Old Female
Perivascular epithelioid cell tumors are rare mesenchymal tumors arising from histologically and immunohistochemically distinctive perivascular epithelioid cells that express both myogenic and melanocytic markers. These tumors are known to arise from different organs in the body and usually have an...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4119907/ https://www.ncbi.nlm.nih.gov/pubmed/25114821 http://dx.doi.org/10.1155/2014/534175 |
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author | Kapur, Sakshi Patel, Napoleon K. Levin, Miles B. Huang, Richard |
author_facet | Kapur, Sakshi Patel, Napoleon K. Levin, Miles B. Huang, Richard |
author_sort | Kapur, Sakshi |
collection | PubMed |
description | Perivascular epithelioid cell tumors are rare mesenchymal tumors arising from histologically and immunohistochemically distinctive perivascular epithelioid cells that express both myogenic and melanocytic markers. These tumors are known to arise from different organs in the body and usually have an unpredictable clinical course. We report a case of a 49-year-old female who presented with diffuse abdominal pain, fever, chills, and nonbilious vomiting for a day. Work-up revealed a mesenteric mass measuring 13.5 × 7.7 × 9.5 cm, arising in the mesentery of the hepatic flexure, with adjacent gas suggestive of fistularization into the right colon. An exploratory laparotomy with resection of the mesenteric mass was performed, and the initial histopathology results were compatible with either an adenocarcinoma or a sarcoma; however, because of poor differentiation it was difficult to make a definitive diagnosis. However, final histopathology results revealed a malignant perivascular epithelioid cell tumor (with reservation that a S100 negative metastatic melanoma must be excluded clinically). Following surgery the patient was started on everolimus, an m-TOR inhibitor, and has shown good response to this medication. |
format | Online Article Text |
id | pubmed-4119907 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-41199072014-08-11 Malignant Mesenteric Perivascular Epithelioid Cell Neoplasm Presenting as an Intra-Abdominal Fistula in a 49-Year-Old Female Kapur, Sakshi Patel, Napoleon K. Levin, Miles B. Huang, Richard Case Rep Oncol Med Case Report Perivascular epithelioid cell tumors are rare mesenchymal tumors arising from histologically and immunohistochemically distinctive perivascular epithelioid cells that express both myogenic and melanocytic markers. These tumors are known to arise from different organs in the body and usually have an unpredictable clinical course. We report a case of a 49-year-old female who presented with diffuse abdominal pain, fever, chills, and nonbilious vomiting for a day. Work-up revealed a mesenteric mass measuring 13.5 × 7.7 × 9.5 cm, arising in the mesentery of the hepatic flexure, with adjacent gas suggestive of fistularization into the right colon. An exploratory laparotomy with resection of the mesenteric mass was performed, and the initial histopathology results were compatible with either an adenocarcinoma or a sarcoma; however, because of poor differentiation it was difficult to make a definitive diagnosis. However, final histopathology results revealed a malignant perivascular epithelioid cell tumor (with reservation that a S100 negative metastatic melanoma must be excluded clinically). Following surgery the patient was started on everolimus, an m-TOR inhibitor, and has shown good response to this medication. Hindawi Publishing Corporation 2014 2014-07-10 /pmc/articles/PMC4119907/ /pubmed/25114821 http://dx.doi.org/10.1155/2014/534175 Text en Copyright © 2014 Sakshi Kapur et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Kapur, Sakshi Patel, Napoleon K. Levin, Miles B. Huang, Richard Malignant Mesenteric Perivascular Epithelioid Cell Neoplasm Presenting as an Intra-Abdominal Fistula in a 49-Year-Old Female |
title | Malignant Mesenteric Perivascular Epithelioid Cell Neoplasm Presenting as an Intra-Abdominal Fistula in a 49-Year-Old Female |
title_full | Malignant Mesenteric Perivascular Epithelioid Cell Neoplasm Presenting as an Intra-Abdominal Fistula in a 49-Year-Old Female |
title_fullStr | Malignant Mesenteric Perivascular Epithelioid Cell Neoplasm Presenting as an Intra-Abdominal Fistula in a 49-Year-Old Female |
title_full_unstemmed | Malignant Mesenteric Perivascular Epithelioid Cell Neoplasm Presenting as an Intra-Abdominal Fistula in a 49-Year-Old Female |
title_short | Malignant Mesenteric Perivascular Epithelioid Cell Neoplasm Presenting as an Intra-Abdominal Fistula in a 49-Year-Old Female |
title_sort | malignant mesenteric perivascular epithelioid cell neoplasm presenting as an intra-abdominal fistula in a 49-year-old female |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4119907/ https://www.ncbi.nlm.nih.gov/pubmed/25114821 http://dx.doi.org/10.1155/2014/534175 |
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