Abstract
A 71-year-old man with multiple hepatic cysts was referred to our hospital because of a solitary liver tumor. Abdominal CT scan revealed a solid 40-mm mass in hepatic segments 4 and 5 with heterogeneous enhancement of its marginal lesion. Under the diagnosis of hilar cholangiocarcinoma, he underwent surgical resection by central bisegmentectomy with caudate lobe and extrahepatic bile duct resection and reconstruction by intrahepaticojejunostomy.
Pathological analysis revealed that the tumor predominantly comprised severely atypical spindle-shaped cells with fascicular pattern and severe perineural invasion. Immunohistochemical analysis revealed that the tumor was positive for S-100 and vimentin and negative for CD34, c-kit, desmin, and Smooth muscle actin (SMA), typical for a malignant peripheral nerve sheath tumor (MPNST). So far, he has survived for 12 months without recurrence.
MPNSTs are relatively rare soft tissue sarcomas that develop from Schwann cells. They account for approximately 5% of all soft tissue sarcomas. An MPNST developing in the hepatic portal region is very rare. We report a case of a patient successfully treated with aggressive surgical resection.
