| 16. Neoplasms of the
Liver / L.J. Worobetz |
page 550 |
Neoplasms of the liver can be divided into
benign and malignant, with malignant tumors being either primary or secondary from a
cancer that has metastasized from elsewhere. The sites from which metastases occur
frequently include lung, colon, pancreas, breast, stomach and ovary. In North America,
primary hepatic neoplasms are uncommon and metastatic tumors commonly seen, whereas
worldwide, especially in areas such as the Far East, hepatocellular carcinoma (HCC) is
much more prevalent than metastatic disease.
| 16.1 Benign Tumors of
the Liver |
page 550 |
Benign tumors of in the liver are detected
more frequently than in the past, in part as a result of the increased use of diagnostic
imaging tests, including ultrasound and CT scanning, for reasons unrelated to the tumors.
Benign tumors are divided into three categories: hepatocellular, cholangiocellular and
non-epithelial.
Hepatocellular adenomas are benign tumors seen primarily in females in
their third and fourth decades. Their increased prevalence paralleled the introduction of
the oral contraceptive, suggesting a hormonal influence in their pathogenesis. The tumors
may be multiple, usually involving the right lobe, and may be > 10 cm in diameter.
Clinical features include an asymptomatic presentation, pain, palpable mass or features of
hemorrhage into the tumor. Diagnosis is usually established with imaging techniques
including ultrasound, CT scanning, angiography and radionuclear scanning. The tumor is
often hypervascular but with hypovascular areas. The risk of malignancy is 10% with an
increased risk in the larger, multiple tumors. Management includes stopping oral
contraceptives and resection of larger, symptomatic tumors.
Focal nodular hyperplasia
differs from the adenoma in that this solid tumor has a fibrous core and includes other
cell types such as atypical hepatocytes, Kupffer's cells and inflammatory cells. The
lesion is vascular and because of the presence of Kupffer's cells will appear as an area
of increased uptake on radionuclear scanning. Although also more common in women, it does
not appear related to the oral contraceptive. No treatment is needed if the patient is
asymptomatic.
Hemangiomas of the liver are seen in about 0.5-7% of the general population.
These are usually detected by imaging techniques done for other reasons. These vascular
lesions are usually asymptomatic and are more common in women. The lesion does not require
any treatment as there is no malignant potential and hemorrhage is rare. The vascular
nature can be confirmed by red blood cell-labeled nuclear scanning.
| 16.2 Primary
Hepatocellular Carcinoma (HCC) |
page 551 |
Although HCC is one of the most common
tumors worldwide, it represents only 1-2% of malignant tumors in North America. The tumor
is more commonly seen in males and usually arises from areas of long-standing liver injury
with liver necrosis and repair, as in the cirrhotic liver. The risk of developing HCC is
greatest in patients with chronic hepatitis B and C infections, hemochromatosis and
cirrhosis from a1-antitrypsin deficiency. Hepatitis B can predispose to hepatoma in either
a cirrhotic or noncirrhotic liver by the integration of the hepatitis B-DNA into portions
of the human genome within the hepatocytes where growth-modifying genes are present.
Recent studies suggest that in cases of HCC, HCV is substantially more common than HBV.
HCC is not often seen in patients with alcoholic cirrhosis who continue to drink, unless
the alcoholic has coexisting viral hepatitis. Hepatic co-carcinogens that appear to play a
role in HCC include aflatoxin and vinyl chloride.
The clinical recognition of HCC may be
difficult, as the tumors often occur in patients with underlying cirrhosis, and the signs
and symptoms may suggest progression of underlying liver disease. The more common
presentations include deterioration of the known cirrhotic with rapid weight loss, an
enlarging liver with a mass, abdominal pain or bloody ascites. A friction rub or bruit may
be present over the liver. Patients may present with one of the many paraneoplastic
syndromes, including erythrocytosis, hypercalcemia, dysproteinemia or hypoglycemia. Serum
a-fetoprotein levels are greater than 500 µg/L in 70-80% of cases. Ultrasound and CT scan
imaging are often used to identify the mass, but occasionally radionuclear scanning (using
gallium) or x-ray angiography may help further define the nature of the mass lesion and
provide important operative details. A biopsy of the mass or examination of the ascitic
fluid for cytology may yield a conclusive diagnosis.
Treatment is disappointing. Surgical
resection represents the only opportunity for cure, but resection is often complicated by
the presence of underlying cirrhosis, the occasional multicentric nature of the tumor and
the presence of micrometastases. Up to 70% of patients already have metastatic disease at
the time of diagnosis. The mean survival from the time of diagnosis is approximately 6 to
12 months. Liver transplantation may be considered, but recurrence of tumor and presence
of metastases after transplantation have limited its usefulness. Other experimental
approaches include hepatic artery embolization with chemotherapy, immunotherapy with
monoclonal antibodies tagged with chemotherapy and gene therapy with cytotoxic
agents.
Screening strategies include ultrasound and/or a-fetoprotein determinations every 6
months in high-risk patients. This approach clearly identifies tumors at an earlier stage
but has not yet been shown to improve morbidity or mortality rates. |