2020 Volume 56 Issue 4 Pages 407-413
We herein present the case of a giant hepatic tumor in a 14-year-old girl, who had received ulcerative colitis treatment since 13 years of age. Ultrasonography (US) was carried out as a routine work-up examination because she sometimes complained of a dull abdominal pain. US showed a huge parenchymal tumor in the lateral segment of the hepatic left lobe, followed by CT and magnetic resonance imaging. These imaging modalities showed that the blood flow of the dilated left hepatic artery around the tumor returned to the left hepatic vein (early venous return). Although central scars suggested the possibility of focal nodular hyperplasia (FNH), early venous return and the absence of a spoke-wheel appearance suggested the possibility of angiomyolipoma. We successfully performed left lateral segmentectomy, which resulted in the disappearance of the abdominal pain. The histopathologic findings of the resected specimen revealed a fibrous central scar and an abnormal portal region, showing a map-like expression pattern of glutamine synthetase revealed by immunohistochemical staining. Therefore, the hepatic tumor was finally diagnosed as FNH, which is the second most common benign pediatric tumor of the liver, and the portal regional malformation was suggested as a potential source of tumorigenesis. Although the central scar and spoke-wheel appearance on imaging modalities are specific for FNH, we are not always able to obtain these findings, leading to the possible misdiagnosis of other hepatic tumors. If we suspect that the tumor is FNH, we should consider surgical intervention for cases with the clinical symptoms of FNH, the tendency of the tumor to grow or atypical imaging findings.
