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.