Tumours of the
bile duct
Papillomatosis
This rare condition is characterised by the presence of multiple
mucoussecreting tumours of the biliary epithelium. These tumours are low-grade
papillary carcinomas and should be treated by choledochoscopy with obliteration
of the papillary lesions. The malignancy tends to develop over a period of years
and may require liver resection. If both lobes are affected then liver
transplantation may be required.
Carcinoma
of the bile duct
Incidence
and aetiology
The incidence of bile-duct carcinoma varies markedly in different
parts of the world. It is very rare in the UK, but high rates are found in parts
of the world where biliary infection is common. It is associated with the
presence of bile-duct stones in a quarter of patients, and is more frequent in
patients with sclerosing cholangitis and ulcerative colitis. Anatomical
abnormalities, particularly intrahepatic cystic change and choledochal cyst, are
also associated with an increased incidence. Clinorchis sinensis is a recognised
aetiological factor.
Pathology
Macroscopically, the tumour arises as a small nodule which rapidly
encases the bile duct causing jaundice. The tumour invades locally spreading via
the nerves, the lymphatics and the portal venous trunk. It may directly invade
the portal vein or the surrounding liver. Microscopically, the majority of bile
duct tumours are well to poorly differentiated adenocarcinomas.
Clinical
features
The patient presents with jaundice. The diagnosis is suspected when
obstruction in the biliary tree with a characteristic stricture is demonstrated.
Local spread is frequent and metastases are
common at the time of presentation. The diagnosis is confirmed by biopsy, either
by the percutaneous transhepatic route or endoscopically, or by cytological
brushings taken at endoscopy.
If the patient is under 70 years of age and
the tumour is localised either to one lobe of the liver or to the extrahepatic
bile duct then resection is possible in 10—20 per cent of patients. Such
resections may involve excision of a lobe of the liver and reconstruction of the
biliary tree. With the improved techniques associated with modern liver surgery
the mortality is now 5 per cent. The
median survival is 18 months, with 20 per cent of the patients surviving for 5
years.
Carcinoma
of the gall bladder
This is rare in the Western world but there are areas where the
incidence is high (in Patna, India, the incidence reaches as high as 9 per cent
of biliary tract disease). The tumour is found in less than I per cent of
gall-bladder operations. In over 90 per cent of instances gallstones are
present. The patients are usually in their late 70s, with a female to male ratio
of 5:1. The tumour is usually scirrhous, but squamous cell and mixed squamous
adenocarcinomas are found. Spread is by direct invasion through the mucosa to
the serosa and into the liver, the lymphatics and the veins.
Clinical
features
Most present either with an extensive mass in the liver during
investigations for jaundice, or at cholecystectomy at the time the histology is
received, the tumour being unrecognised by the surgeon.
Treatment
and prognosis
Those that are diagnosed at cholecystectomy and confined to the mucosa
have a good prognosis. It is debated whether such patients should have a wide
excision with resection of adjacent liver and lymph nodes. For those tumours
that involve the serosa, the prognosis is poor and chemoradiotherapy is all that
can be offered. The survival rate is less than 5 per cent at 5 years and
the median survival is 12 months.