Hepatic MPNST without NF1: normal tumor markers, no recurrence 2 years after resectionCase Report Shows Surgery for Rare Liver Tumor Success

This is a case report and literature review of a 56-year-old woman with hepatic malignant peripheral nerve sheath tumor (MPNST) in the absence of neurofibromatosis type 1. The tumor presented as a hypovascular hepatic lesion on contrast-enhanced ultrasound and computed tomography, with serum tumor markers within normal ranges. The patient underwent radical tumor resection and showed no signs of recurrence or metastasis at approximately 2 years of follow-up.

The review synthesizes available literature on imaging features of hepatic MPNST, noting that contrast-enhanced ultrasound and contrast-enhanced computed tomography manifestations are non-specific and require differentiation from other liver malignancies such as hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and hepatic angiosarcoma. The authors highlight that hepatic MPNST presents with non-specific clinical features and normal tumor markers, making preoperative diagnosis challenging.

Key limitations include the small sample size (single case report) and limited available data on this rare entity. The evidence is insufficient to guide management beyond surgical resection. Practice relevance is confined to raising awareness of this rare tumor in the differential diagnosis of liver masses, especially when tumor markers are normal and imaging shows a hypovascular lesion.