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Are liver nested stromal epithelial tumors always low aggressive?

Nested stromal-epithelial tumor (NSET) is a non-hepatocytic and non-biliary tumor of the liver consisting of nests of epithelial and spindled cells with associated myofibroblastic stroma and variable intra-lesional calcification and ossification, which represents a very rare and challenging disease....

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Autores principales: Meletani, Tania, Cantini, Luca, Lanese, Andrea, Nicolini, Daniele, Cimadamore, Alessia, Agostini, Andrea, Ricci, Giulia, Antognoli, Stefania, Mandolesi, Alessandra, Guido, Maria, Alaggio, Rita, Giuseppetti, Gian Marco, Scarpelli, Marina, Vivarelli, Marco, Berardi, Rossana
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5739931/
https://www.ncbi.nlm.nih.gov/pubmed/29290661
http://dx.doi.org/10.3748/wjg.v23.i46.8248
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author Meletani, Tania
Cantini, Luca
Lanese, Andrea
Nicolini, Daniele
Cimadamore, Alessia
Agostini, Andrea
Ricci, Giulia
Antognoli, Stefania
Mandolesi, Alessandra
Guido, Maria
Alaggio, Rita
Giuseppetti, Gian Marco
Scarpelli, Marina
Vivarelli, Marco
Berardi, Rossana
author_facet Meletani, Tania
Cantini, Luca
Lanese, Andrea
Nicolini, Daniele
Cimadamore, Alessia
Agostini, Andrea
Ricci, Giulia
Antognoli, Stefania
Mandolesi, Alessandra
Guido, Maria
Alaggio, Rita
Giuseppetti, Gian Marco
Scarpelli, Marina
Vivarelli, Marco
Berardi, Rossana
author_sort Meletani, Tania
collection PubMed
description Nested stromal-epithelial tumor (NSET) is a non-hepatocytic and non-biliary tumor of the liver consisting of nests of epithelial and spindled cells with associated myofibroblastic stroma and variable intra-lesional calcification and ossification, which represents a very rare and challenging disease. Most of the reported cases have been treated with surgery, obtaining a long survival outcome. Here, we report the case of a 31-year-old Caucasian man who underwent surgery at our institution for a large, lobulated, multinodular mass of the right hemi-liver. The histological exam confirmed the diagnosis of NSET. After 6 mo from surgery, a liver recurrence was described and a chemo-embolization was performed. After a further disease progression, based on the correlation between the histological features of the disease and those of the hepatoblastoma, a similar chemotherapy regimen (with cisplatin and ifosfamide/mesna chemotherapy, omitting doxorubicin due to liver impairment) was administered. However, infection of the biliary catheter required a dose modification of the treatment. No benefit was noted and a progression of disease was radiologically assessed after only four cycles. The worsening of the clinical status prevented further treatments, and the patient died a few months later. This case report documents how the NSET might have an aggressive and non-preventable behavior. No chemotherapy schedules with a proved efficacy are available, and new data are needed to shed light on this rare neoplasm.
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spelling pubmed-57399312017-12-30 Are liver nested stromal epithelial tumors always low aggressive? Meletani, Tania Cantini, Luca Lanese, Andrea Nicolini, Daniele Cimadamore, Alessia Agostini, Andrea Ricci, Giulia Antognoli, Stefania Mandolesi, Alessandra Guido, Maria Alaggio, Rita Giuseppetti, Gian Marco Scarpelli, Marina Vivarelli, Marco Berardi, Rossana World J Gastroenterol Case Report Nested stromal-epithelial tumor (NSET) is a non-hepatocytic and non-biliary tumor of the liver consisting of nests of epithelial and spindled cells with associated myofibroblastic stroma and variable intra-lesional calcification and ossification, which represents a very rare and challenging disease. Most of the reported cases have been treated with surgery, obtaining a long survival outcome. Here, we report the case of a 31-year-old Caucasian man who underwent surgery at our institution for a large, lobulated, multinodular mass of the right hemi-liver. The histological exam confirmed the diagnosis of NSET. After 6 mo from surgery, a liver recurrence was described and a chemo-embolization was performed. After a further disease progression, based on the correlation between the histological features of the disease and those of the hepatoblastoma, a similar chemotherapy regimen (with cisplatin and ifosfamide/mesna chemotherapy, omitting doxorubicin due to liver impairment) was administered. However, infection of the biliary catheter required a dose modification of the treatment. No benefit was noted and a progression of disease was radiologically assessed after only four cycles. The worsening of the clinical status prevented further treatments, and the patient died a few months later. This case report documents how the NSET might have an aggressive and non-preventable behavior. No chemotherapy schedules with a proved efficacy are available, and new data are needed to shed light on this rare neoplasm. Baishideng Publishing Group Inc 2017-12-14 2017-12-14 /pmc/articles/PMC5739931/ /pubmed/29290661 http://dx.doi.org/10.3748/wjg.v23.i46.8248 Text en ©The Author(s) 2017. 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
Meletani, Tania
Cantini, Luca
Lanese, Andrea
Nicolini, Daniele
Cimadamore, Alessia
Agostini, Andrea
Ricci, Giulia
Antognoli, Stefania
Mandolesi, Alessandra
Guido, Maria
Alaggio, Rita
Giuseppetti, Gian Marco
Scarpelli, Marina
Vivarelli, Marco
Berardi, Rossana
Are liver nested stromal epithelial tumors always low aggressive?
title Are liver nested stromal epithelial tumors always low aggressive?
title_full Are liver nested stromal epithelial tumors always low aggressive?
title_fullStr Are liver nested stromal epithelial tumors always low aggressive?
title_full_unstemmed Are liver nested stromal epithelial tumors always low aggressive?
title_short Are liver nested stromal epithelial tumors always low aggressive?
title_sort are liver nested stromal epithelial tumors always low aggressive?
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5739931/
https://www.ncbi.nlm.nih.gov/pubmed/29290661
http://dx.doi.org/10.3748/wjg.v23.i46.8248
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