Cancer of the bile ducts associated with ulcerative colitis

Thirteen patients with bile duct cancer (excluding gallbladder) and associated chronic ulcerative colitis (CUC) were seen at the Mayo Clinic from 1935 through 1973. Most patients had initial symptoms of severe diarrhea and bleeding, followed by a pattern of mild-to-moderate disease with exacerbations and remissions. Three patients had especially severe symptoms and underwent total colectomy (1 patient) or proctocolectomy (2 patients) an average of 15.7 years from onset of CUC symptoms. Anorexia, followed rapidly by the development of progressive jaundice (or a sudden deterioration when liver disease was already present), marked the onset of symptoms of bile duct cancer in the 13 patients. The overall mean duration from onset of CUC to development of symptoms of bile duct cancer was 19 years. The patients in whom colitis was managed by proctocolectomy or total abdominal colectomy developed symptoms of bile duct cancer an average of 9.4 years after colectomy. When cancer was diagnosed, the tumor had spread beyond the bile ducts in 10 patients. The tumors were difficult to identify and often infiltrated the hepatic hilus. The present series and review of the literature suggest that the relationship between CUC and bile duct cancer is more than a chance occurrence. The carcinoma has an onset approximately 3 decades earlier than does carcinoma of the bile ducts without CUC. Surgical removal of the diseased colon and mode of medical management of the unresected colon have no relationship to the subsequent development of carcinoma of the bile ducts; neither does the extent or severity of the colonic disease. The prognosis of carcinoma of the bile ducts unfortunately continues to be dismal.