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 Hepatocellular Carcinoma Hepatocellular carcinoma (HCC) usually occurs in patients with cirrhosis and is common in areas where infection with hepatitis B and C viruses is prevalent. Symptoms and signs are usually nonspecific... read more , 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 Primary Sclerosing Cholangitis (PSC) Primary sclerosing cholangitis (PSC) is patchy inflammation, fibrosis, and strictures of the bile ducts that has no known cause. However, 80% of patients with PSC also have inflammatory bowel... read more greatly increases risk of cholangiocarcinoma (1 Reference Primary liver cancer is usually hepatocellular carcinoma (HCC). The first manifestations of liver cancer are usually nonspecific, delaying the diagnosis. When diagnosed at advanced stages, prognosis... read more ). 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 Liver Transplantation Liver transplantation is the 2nd most common type of solid organ transplantation. (See also Overview of Transplantation.) Indications for liver transplantation include Cirrhosis (70% of transplantations... read more 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 Familial Adenomatous Polyposis Familial adenomatous polyposis is a hereditary disorder causing numerous colonic polyps and frequently results in colon carcinoma, often by age 40. Patients are usually asymptomatic but may... read more . 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 Imaging Tests of the Liver and Gallbladder Imaging is essential for accurately diagnosing biliary tract disorders and is important for detecting focal liver lesions (eg, abscess, tumor). It is limited in detecting and diagnosing diffuse... read more 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.