| 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) |
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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. |