Primary adenocarcinoma of the bile ducts. Clinical characteristics and natural history.

Primary bile duct carcinoma is a malignancy with a poor prognosis, but recent diagnostic developments allow earlier detection and possibly improved chances for effective palliation or surgical cure. In order to increase the clinical understanding and awareness of this disorder, 43 patients with primary adenocarcinoma of the bile ducts were reviewed. The mean patient age was 63, and symptoms of nausea, abdominal pain, and pruritus were reported in a majority of patients. Documented weight loss, alcoholic stools, cutaneous icterus, and hepatomegaly were each present in a majority of patients. Serum bilirubin and alkaline phosphatase determinations were abnormal in 40 of 43 patients (93%), and cholangiography was the diagnostic study providing the most discriminating information. Locally invasive disease and biliary obstruction was the major cause of morbidity and mortality, and there was only one surgical cure. These data suggest that cholangiography and nonsurgical techniques for biliary drainage should be employed in most patients who are not optimal surgical candidates.