Fibrolamellar hepatocellular carcinoma: Pre- and posttherapy evaluation with CT and MR imaging

PURPOSE: To determine the features of advanced hepatic and extrahepatic fib rolamellar hepatocellular carcinomas (HCCs) and their effects on immediate surgical management and tumor recurrence. MATERIALS AND METHODS: Thirty-one patients with fibrolamellar HCC underwent pretherapy computed tomography (CT); 11 underwent pretherapy magnetic reso nance (MR) imaging. All 40 patients underwent posttherapy CT; four, follow- up MR imaging. Imaging, surgical, and histopathologic findings were correla ted. RESULTS: Twenty-five (81%) patients had solitary tumors (mean maximum diame ter, 13 cm). Thirteen (42%) patients had intrahepatic biliary obstruction; 27 (87%) patients had involvement of the portal or hepatic veins. Thirteen (42%) had extrahepatic tumor spread, nine (29%) had distant metastases on p retherapy images, and 20 (65%) had lymphadenopathy. Thirty-two (80%) of 40 patients underwent exploration surgery; curative resection was attempted in 25 (62%), including four patients who underwent liver transplantation. Onl y 17 patients were considered to have had hepatic and extrahepatic tumors c ompletely excised. Tumor recurred in all eight of the 17 patients who had e xtrahepatic disease at pretherapy CT and in four of the seven patients who seemed to have tumor limited to the liver. A combination of repeat tumor re section and adjuvant chemotherapy resulted in prolonged tumor-free survival in some cases, CONCLUSION: Fibrolamellar HCC frequently demonstrates aggressive local inva sion and nodal and distant metastases. Pretherapy and follow-up imaging are important for staging, surveillance, and optimal management. Aggressive su rgical resection may be helpful to control fibrolamellar HCC and to prolong survival in appropriately selected cases.