Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Kapur, Sakshi, Patel, Napoleon K., Levin, Miles B., Huang, Richard
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2014
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
_version_ 1782329015296589824
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
work_keys_str_mv AT kapursakshi malignantmesentericperivascularepithelioidcellneoplasmpresentingasanintraabdominalfistulaina49yearoldfemale
AT patelnapoleonk malignantmesentericperivascularepithelioidcellneoplasmpresentingasanintraabdominalfistulaina49yearoldfemale
AT levinmilesb malignantmesentericperivascularepithelioidcellneoplasmpresentingasanintraabdominalfistulaina49yearoldfemale
AT huangrichard malignantmesentericperivascularepithelioidcellneoplasmpresentingasanintraabdominalfistulaina49yearoldfemale