Case Report
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Dec 14, 2017; 23(46): 8248-8255
Published online Dec 14, 2017. doi: 10.3748/wjg.v23.i46.8248
Are liver nested stromal epithelial tumors always low aggressive?
Tania Meletani, Luca Cantini, Andrea Lanese, Daniele Nicolini, Alessia Cimadamore, Andrea Agostini, Giulia Ricci, Stefania Antognoli, Alessandra Mandolesi, Maria Guido, Rita Alaggio, Gian Marco Giuseppetti, Marina Scarpelli, Marco Vivarelli, Rossana Berardi
Tania Meletani, Luca Cantini, Andrea Lanese, Giulia Ricci, Stefania Antognoli, Rossana Berardi, Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
Daniele Nicolini, Marco Vivarelli, Hepatobiliary and Transplantation Surgery, Department of Clinical and Experimental Medicine, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
Alessia Cimadamore, Alessandra Mandolesi, Marina Scarpelli, Section of Pathological Anatomy, Department of Biomedical Sciences and Public Health, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
Andrea Agostini, Gian Marco Giuseppetti, Department of Radiology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
Maria Guido, Rita Alaggio, Pathology Department, Padova University, 35121 Padova, Italy
Author contributions: Meletani T, Cantini L, Lanese A, Giuseppetti GM, Vivarelli M and Berardi R designed the report; Cimadamore A, Mandolesi A, Giudo M, Alaggio R and Scarpelli M performed the pathological analyses; Ricci G and Antognoli S collected the patient’s clinical data; Meletani T, Cantini L, Lanese A, Nicolini D, Cimadamore A and Agostini A analyzed the data and wrote the paper.
Institutional review board statement: The study was reviewed and approved by the local Ethical Committee.
Informed consent statement: The patient involved in this study gave verbal informed consent authorizing the use and disclosure of his protected health information.
Conflict-of-interest statement: All authors declare having no conflicting interest.
Open-Access: 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. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Rossana Berardi, MD, Head of Department of Medical Oncology, Director of the Postgraduate School of Medical Oncology, Director of “Genetic Cancer” Center, Deputy Director of Department of Clinical and Molecular Science, University Hospital Ospedali Riuniti Umberto I - GM Lancisi - G Salesi and Polytechnic University Ancona, via conca 71, 60126 Marche, Italy. r.berardi@univpm.it
Telephone: +39-71-5964169 Fax: +39-71-65964192
Received: June 20, 2017
Peer-review started: June 22, 2017
First decision: July 13, 2017
Revised: July 27, 2017
Accepted: August 25, 2017
Article in press: August 25, 2017
Published online: December 14, 2017
Processing time: 174 Days and 16.7 Hours
Abstract

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.

Keywords: Nested stromal epithelial tumor; Liver; Rare; Chemotherapy; Aggressive; Metastatic

Core tip: Nested stromal-epithelial tumor (NSET) of the liver is a very rare type of cancer, few cases have been reported in the world. Most of the literature described a low tendency of relapse, the majority of the reported cases have been treated with surgery, obtaining a long survival outcome. Our patient developed a more aggressive disease, which relapsed soon after surgery, and progressed after first line chemotherapy. We aimed to update the literature about NEST, especially in patients with either metastatic or recurrent disease, for whom no standard treatment is currently available.