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Primary Liver Cancer

by Steven K. Herrine, MD

Primary liver cancer is usually hepatocellular carcinoma (see Hepatocellular Carcinoma). The first manifestations of liver cancer are usually nonspecific, delaying the diagnosis. Prognosis is usually poor.

Other Primary Liver Cancers

Other primary liver cancers are uncommon or rare. Diagnosis usually requires biopsy. Prognosis is typically poor.

Some cancers, if localized, can be resected. Resection or liver transplantation may prolong survival.

Fibrolamellar carcinoma

This distinct variant of hepatocellular carcinoma 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, HBV, HCV, or other known risk factors. α-Fetoprotein (AFP) levels are rarely elevated.

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


Cholangiocarcinoma

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 hepatocellular carcinoma (see Hepatocellular Carcinoma); histologically, the two may overlap. Primary sclerosing cholangitis greatly increases risk of cholangiocarcinoma.


Hepatoblastoma

Although rare, hepatoblastoma is one of the most common primary liver cancers in infants, particularly those with a family history of familial adenomatous polyposis (see 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.


Angiosarcoma

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


Cystadenocarcinoma

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

Treatment is liver resection.


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