BILIARY-TRACT CANCER ACCOMPANIED BY ANOMALOUS JUNCTION OF PANCREATICOBILIARY DUCTAL SYSTEM IN ADULTS

BACKGROUND: Anomalous junction of the pancreaticobiliary ductal system (AJPBDS) is a congenital anomaly in which the junction is located out side the duodenal wall. Recently, attention has been focused on the hi gh incidence of malignancy in this anomaly. The purpose of this study was to clarify the clinicopathological features of this anomaly and to determine the appropriate surgical approach for biliary tract cancer associated with AJPBDS. METHODS: The data for 38 patients with AJPBDS, including 14 who had been treated for biliary tract cancer (2 with bi le duct cancer and 12 with gallbladder cancer), were retrospectively r eviewed. We assessed the clinical features, characteristics of the tum or, operative procedure, and outcome for each patient. RESULTS: The in cidence of malignancy in AJPBDS was 17.8% (2 patients with bile duct c ancer and 3 with gallbladder cancer) in the bile duct dilatation group (n = 28) and 90% (9 patients with gallbladder cancer) in the no-dilat ation group (n = 10). The mean length of the common channel was 24.7 m m (range 20 to 35 mm). Resection with lymphadenectomy was performed in 9 (64.3%) of 14 patients, and curative resection in 5 of these 9 pati ents. Ten (71%) of the 14 patients had lymph node involvement noted ei ther at the time of initial diagnosis or at surgery. The incidence of lymph node metastasis was closely related to the depth of tumor involv ement. Ten patients died of recurrence or primary cancer, from 3 to 30 months after operation. Four patients are still alive without recurre nt disease from 2.5 to 13 years after operation. CONCLUSION: For patie nts with AJPBDS without bile duct dilatation, prophylactic cholecystec tomy is recommended even if no malignant lesion is found in the gallbl adder because of the high incidence of gallbladder cancer and the poor prognosis. Both early detection and curative resection of the tumor a re essential for successful treatment of biliary tract cancer. (C) 199 8 by Excerpta Medica, Inc.