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214 Chapter 5: Hepatic, biliary and pancreatic systems
Pathophysiology Prognosis
Tumour growth causes further impairment of liver func- Primary hepatocellular tumour is a rapidly growing tu-
tion in already cirrhotic patients. Sometimes rare syn- mour,whichusuallypresentslateinpatientswhoalready
dromes occur such as hypercalcaemia, hypoglycaemia haveaseriousunderlyingpathology,cirrhosis.Theprog-
and porphyria cutanea tarda (bullae on the skin follow- nosis is very poor. Median survival is <6 months from
ing sun exposure or minor trauma due to a defect in a diagnosis.
hepatic enzyme).
The tumour marker alpha-fetoprotein (αFP) is nor-
mallysynthesisedbythefetalliver,butinadultsispresent Metastatic liver tumours
at very low levels (<10 ng/mL). When the tumour se-
Definition
cretes αFP, as it does in most cases, it can reach very high
Secondary tumours in the liver are very common.
levels. It is also raised in germ cell tumours (testicular
and ovarian carcinoma).
Aetiology
The most common sites of the primary tumour are
Clinical features
bronchus, breast, bowel (stomach, colon) and pancreas.
Insidious onset with anorexia, weight loss and poorly
The liver is also an important site of growth for lym-
localised upper right quadrant abdominal pain. On ex-
phomas and leukaemias.
amination, the liver is usually enlarged and there may be
an arterial bruit.
Pathophysiology
Macroscopy/microscopy Haematogenous spread via the portal vein or the hep-
The right lobe is more frequently affected than the left. atic artery. One or more tumours may develop. Large or
There is usually one large, haemorrhagic, soft mass or multifocal tumours cause loss of liver parenchyma and
multifocal nodules. Sometimes the tumour is diffusely liver impairment. Liver metastasis may be the first sign
infiltrating with invasion of veins. Histologically cells that there is a primary tumour present, or it may occur in
rangefromwelldifferentiatedtoanaplastic,withatypical a patient with known past or present malignant disease.
nuclear cytology and abnormal architecture.
Clinical features
Complications
Insidious onset of fatigue, anorexia and weight loss oc-
Metastases most commonly occur via the bloodstream
curs. Jaundice is a late sign. Pain and tenderness may
to the lungs. Direct spread may also occur to abdominal
be felt over the liver, which may be irregular, firm and
lymph nodes and to other abdominal organs.
enlarged.
Investigations
Persistently high levels of serum α feto-protein is very
Investigations
Liver function tests may be abnormal. Ultrasound or CT
suspicious of carcinoma.
abdomen may demonstrate the tumours, guided biopsy
Ultrasound and CT abdomen are used to image tu-
may be required if the diagnosis is unclear or if the pri-
mours.
mary tumour is unknown.
Definitive diagnosis is by liver biopsy.
Management Management
Curative treatment by partial liver resection is feasible Treatment depends on the natural history of the primary
in patients with tumour in only one lobe and with tumour.
sufficient liver functional reserve, i.e. no cirrhosis. Chemotherapy may be effective in certain tumour
Palliative treatment: Analgesia, arterial embolisation typessuch as small cell lung carcinoma, lymphoma
or percutaneous injection of alcohol may also cause and breast cancer. Drugs can be infused through a
tumour reduction and pain relief. catheter into the hepatic artery.