A case of inflammatory pseudotumor of the liver causing elevated serum CA19-9 levels

Inflammatory pseudotumor of the liver is a rare lesion characterized by proliferating fibrovascular tissue admixed with inflammatory cells. A 50-yr-old Japanese man was hospitalized because of upper abdominal pain and high fever. Computed tomography revealed a poorly demarcated, low density mass in the left lobe of the liver, and abnormal laboratory findings included WBC 9340/mm3, CRP 10.5 mg/dl, and marked elevation of CA19-9 to 1167.9 U/ml. Endoscopic retrograde cholangiography showed irregularity of the intrahepatic bile duct of the left lateral segment, and the lateral segmental branches of the portal vein were not visualized on the venous phase of abdominal angiography. Ultrasound-guided liver biopsy was performed, but malignant disease, including intrahepatic cholangiocarcinoma, could not be completely ruled out. The patient underwent left hepatic lobectomy with lymph node dissection. Histopathological examination yielded a definitive diagnosis of inflammatory pseudotumor. The lesion was immunohistochemically stained for CA19-9 by the ABC method, and the biliary epithelium in severely inflamed portal canals was found to be positive. The markedly elevated preoperative level of CA 19-9 decreased to almost within the normal range and the patient remains well 2 yr 9 months after surgery, without any complications.