Abstracts
1998 Digestive Disease Week

#2325

DIAGNOSIS AND MANAGEMENT OF CHOLANGIOCARCINOMA IN PRIMARY SCLEROSING CHOLANGITIS. S.A.Ahrendt, H.A.Pitt, A.Nakeeb, A.S.Klein, K.D.Lillemoe, A.N.Kalloo, and J.L.Cameron. Depts. of Surgery and Medicine, The Johns Hopkins University, Baltimore, MD and Dept. of Surgery, Medical College of Wisconsin, Milwaukee, WI.

Cholangiocarcinoma (CCA) remains difficult to diagnose and is a major cause of mortality in patients with primary sclerosing cholangitis (PSC). Recently, serum carcinoembryonic antigen (CEA) levels and endoscopic brush cytology and/or biopsy (EBC) have been reported to improve diagnostic accuracy in patients with CCA and PSC. Between 1980 and 1997, 25 patients were diagnosed with CCA among 161 patients with PSC. The diagnosis of PSC was made coincident with the diagnosis of CCA in 11 patients and preceded it by a mean of 54 months in the remaining 14 patients. Only 4 patients had cirrhosis at the time of diagnosis of CCA. Symptoms, mean age (48 vs 47 years), Mayo risk score (3.6 ± 0.2 vs. 3.7 ± 0.1), and serum bilirubin
(8 ± 2 vs. 7.3 ± 0.8 mg/dL) were similar among patients with and without CCA, respectively. The incidence of inflammatory bowel disease was higher in patients with CCA (85 % vs. 53 %). Nine patients with CCA were managed with either extrahepatic bile duct resection (n=5), hepatic lobectomy (n=1), or liver transplantation (n=3). The remaining 16 patients were unresectable at presentation (metastases (n=13), advanced cirrhosis (n=2), or locally unresectable tumor (n=1)). The tumor site (hilar/extrahepatic biliary tree (n=19), intrahepatic (n=4), or gallbladder (n=2)) was similar among the two groups. Actuarial survival and the sensitivity of serum CEA and endoscopic brush cytology (EBC) and/or biopsy in the diagnosis of CCA were:

Four patients were managed prior to the diagnosis of CCA with endoscopic balloon dilation of dominant biliary strictures for 22 ± 3 months. Three of these patients had metastatic disease at the time of diagnosis of CCA. In summary, cholangiocarcinoma is a frequent cause of mortality in patients with primary sclerosing cholangitis and often is diagnosed early in the course of the disease. Clinical and laboratory parameters do not differentiate patients with CCA. The sensitivity of both serum CEA and endoscopic biliary cytology in diagnosing cholangiocarcinoma at a resectable stage is low. Surgical resection is associated with a modest chance for long-term survival.

Copyright 1996 - 1998, SSAT, Inc. Revised 29 June 1998.