Are liver nested stromal epithelial tumors always low aggressive?

World J Gastroenterol. 2017 Dec 14;23(46):8248-8255. doi: 10.3748/wjg.v23.i46.8248.

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

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Diagnosis, Differential
  • Fatal Outcome
  • Hepatectomy
  • Hepatoblastoma / diagnosis
  • Hepatoblastoma / pathology
  • Humans
  • Liver / diagnostic imaging
  • Liver / pathology*
  • Liver / surgery
  • Liver Neoplasms / diagnosis
  • Liver Neoplasms / pathology
  • Liver Neoplasms / therapy
  • Male
  • Neoplasm Recurrence, Local / diagnosis
  • Neoplasm Recurrence, Local / pathology*
  • Neoplasm Recurrence, Local / therapy
  • Neoplasms, Complex and Mixed / diagnosis
  • Neoplasms, Complex and Mixed / pathology*
  • Neoplasms, Complex and Mixed / therapy
  • Neoplasms, Glandular and Epithelial / diagnosis
  • Neoplasms, Glandular and Epithelial / pathology*
  • Neoplasms, Glandular and Epithelial / therapy
  • Positron-Emission Tomography
  • Tomography, X-Ray Computed