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Are liver nested stromal epithelial tumors always low aggressive?

World Journal of Gastroenterology 2017年第46期23卷 8248-8255页

作者： 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 Medical Oncology University Hospital and Polytechnic University Ancona Hepatobiliary and Trans-plantation Surgery Department of Clinical and Experimental MedicineUniversity Hospital and Polytechnic University Ancona Section of Pathological Anatomy Department of Biomedical Sciences and Public HealthUniversity Hospital and Polytechnic University Ancona Department of Radiology University Hospital and Polytechnic University Ancona Pathology Department Padova University

Nested stromal-epithelial tumor(NSET) is a nonhepatocytic 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 chemoembolization 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.

关键词： Nested stromal epithelial tumor Liver Rare Chemotherapy Aggressive Metastatic

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