Are liver nested stromal epithelial tumors always low aggressive?

Area of Science:

• Hepatology
• Oncology
• Pathology

Background:

• Nested stromal-epithelial tumor (NSET) is a rare non-hepatocytic, non-biliary liver neoplasm.
• Characterized by epithelial and spindled cells with myofibroblastic stroma, calcification, and ossification.
• Surgical resection is the primary treatment, often yielding long-term survival.

Observation:

• A case of a 31-year-old man with a large NSET in the right hemi-liver is presented.
• Post-surgery, the patient experienced liver recurrence and disease progression despite chemo-embolization.
• Chemotherapy (cisplatin, ifosfamide/mesna) showed no benefit and was complicated by infection.

Findings:

• Histological correlation with hepatoblastoma guided chemotherapy regimen.
• Treatment was limited by liver impairment and catheter infection, necessitating dose modification.
• Radiological assessment confirmed disease progression after four cycles of chemotherapy.

Implications:

• This case suggests NSET can exhibit aggressive and potentially uncontrollable behavior.
• Currently, no established chemotherapy regimens demonstrate efficacy for NSET.
• Further research is crucial to understand and manage this rare liver neoplasm effectively.