Primary Liver Cancer

This distinct variant of HCC has a characteristic morphology of malignant hepatocytes enmeshed in lamellar fibrous tissue. It usually occurs in young adults and has no association with preexisting cirrhosis, hepatitis B virus (HBV), hepatitis C virus (HCV), or other known risk factors. Alpha-fetoprotein (AFP) levels are rarely elevated.

Prognosis is better than that for HCC, and many patients survive several years after tumor resection.

This tumor originates in the biliary epithelium. It is common in China, where underlying infestation with liver flukes is believed to contribute. Elsewhere, it is less common than HCC; histologically, the two may overlap. Primary sclerosing cholangitis greatly increases risk of cholangiocarcinoma (1). Prognosis is poor in the vast majority of patients. However, for the few isolated hilar cholangiocarcinomas < 2 cm that are treated with adjuvant brachytherapy, liver transplantation has an evolving role.

Although rare, hepatoblastoma is one of the most common primary liver cancers in infants, particularly those with a family history of familial adenomatous polyposis. It can also develop in children. Some patients with hepatoblastoma present with precocious puberty caused by ectopic gonadotropin production, but the cancer is usually detected because of deteriorating general health and a right upper quadrant mass. An elevated AFP level and abnormal imaging test results may help in the diagnosis.

This rare cancer is associated with specific chemical carcinogens, including industrial vinyl chloride.

This rare disorder is probably secondary to malignant transformation of a cystadenoma and is often multilobular.

Treatment is liver resection.