Primary malignant peripheral nerve sheath tumor of the liver: a case report and literature review.

Background: Malignant peripheral nerve sheath tumor (MPNST) is a malignant neoplasm originating from Schwann cells or other nerve sheath components. It most commonly arises in the extremities and trunk. Primary hepatic MPNST is extremely rare, and most cases are associated with neurofibromatosis type 1 (NF-1). Case presentation: This study reports a rare case of hepatic MPNST without NF-1 in a 56-year-old female. The patient presented with upper abdominal pain and distention. Serum tumor markers were all within normal ranges. Radiological examination identified a hypovascular hepatic lesion, which was pathologically confirmed as MPNST. Radical tumor resection was performed. The patient has been followed up for approximately 2 years, with no signs of recurrence or metastasis. Conclusion: The clinical manifestation of hepatic MPNST is nonspecific with normal tumor markers. Radiologically, it mainly manifests as a hypovascular tumor, which requires differentiation from hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and hepatic angiosarcoma. With a literature review, our article provides a comprehensive summary of its CEUS and CECT manifestations, thereby supplementing the limited available data on this rare entity.