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Perivascular epithelioid cell tumor outgrowth from the liver

INTRODUCTION: Perivascular epithelioid celltumor (PEComa) is a rare mesenchymal neoplasia and can be found in various body sites. On the other hand, hepatic PEComa is very rare, with only a few studies having reported hepatic malignant PEComa. There is no gold standard regarding the use of diagnosti...

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Detalles Bibliográficos
Autores principales: Kirnap, Mahir, Ozgun, Gonca, Moray, Gokhan, Haberal, Mehmet
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6240724/
https://www.ncbi.nlm.nih.gov/pubmed/30453241
http://dx.doi.org/10.1016/j.ijscr.2018.10.046
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author Kirnap, Mahir
Ozgun, Gonca
Moray, Gokhan
Haberal, Mehmet
author_facet Kirnap, Mahir
Ozgun, Gonca
Moray, Gokhan
Haberal, Mehmet
author_sort Kirnap, Mahir
collection PubMed
description INTRODUCTION: Perivascular epithelioid celltumor (PEComa) is a rare mesenchymal neoplasia and can be found in various body sites. On the other hand, hepatic PEComa is very rare, with only a few studies having reported hepatic malignant PEComa. There is no gold standard regarding the use of diagnostic imaging studies. The diagnosis of hepatic PEComa is made by a positive immunohistochemical staining for HMB45 and Melan A. Herein, we discussed the therapeutic and follow-up process of a symptomatic hepatic PEComa case. PRESENTATION OF CASE: A 22-year-old woman presented with a palpable mass in abdomen. A computerized tomographic examination showed a giant hepatic mass of left lobe origin, which was excised surgically. The pathology result was reported as a PEComa. DISCUSSION: The diagnostic approach, treatment modalities, and follow-up procedures are not standard. The main treatment modality for PEComa is surgical excision with adequate surgical margin. CONCLUSION: A longer follow-up is required for patients with hepatic PEComa because the nature of the disease is not entirely clear.
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spelling pubmed-62407242018-11-29 Perivascular epithelioid cell tumor outgrowth from the liver Kirnap, Mahir Ozgun, Gonca Moray, Gokhan Haberal, Mehmet Int J Surg Case Rep Article INTRODUCTION: Perivascular epithelioid celltumor (PEComa) is a rare mesenchymal neoplasia and can be found in various body sites. On the other hand, hepatic PEComa is very rare, with only a few studies having reported hepatic malignant PEComa. There is no gold standard regarding the use of diagnostic imaging studies. The diagnosis of hepatic PEComa is made by a positive immunohistochemical staining for HMB45 and Melan A. Herein, we discussed the therapeutic and follow-up process of a symptomatic hepatic PEComa case. PRESENTATION OF CASE: A 22-year-old woman presented with a palpable mass in abdomen. A computerized tomographic examination showed a giant hepatic mass of left lobe origin, which was excised surgically. The pathology result was reported as a PEComa. DISCUSSION: The diagnostic approach, treatment modalities, and follow-up procedures are not standard. The main treatment modality for PEComa is surgical excision with adequate surgical margin. CONCLUSION: A longer follow-up is required for patients with hepatic PEComa because the nature of the disease is not entirely clear. Elsevier 2018-11-10 /pmc/articles/PMC6240724/ /pubmed/30453241 http://dx.doi.org/10.1016/j.ijscr.2018.10.046 Text en © 2018 The Author(s) http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Kirnap, Mahir
Ozgun, Gonca
Moray, Gokhan
Haberal, Mehmet
Perivascular epithelioid cell tumor outgrowth from the liver
title Perivascular epithelioid cell tumor outgrowth from the liver
title_full Perivascular epithelioid cell tumor outgrowth from the liver
title_fullStr Perivascular epithelioid cell tumor outgrowth from the liver
title_full_unstemmed Perivascular epithelioid cell tumor outgrowth from the liver
title_short Perivascular epithelioid cell tumor outgrowth from the liver
title_sort perivascular epithelioid cell tumor outgrowth from the liver
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6240724/
https://www.ncbi.nlm.nih.gov/pubmed/30453241
http://dx.doi.org/10.1016/j.ijscr.2018.10.046
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