Carcinoma of the gallbladder associated with anomalous junction of the pancreaticobiliary duct in adults

Background: Anomalous junction of the pancreaticobiliary duct (AJPBD) is a congenital anomaly in which the junction is located outside the sphincter o f Oddi. A high incidence of gallbladder carcinoma (GBC) has been reported i n adult patients with AJPBD. Study Design: Fourteen adult patients with AJPBD and 50 with GBC not associ ated with AJPBD were reviewed retrospectively to identify the clinical char acteristics of AJPBD accompanied by GBC and to clarify the differences in c linicopathologic features between GBC associated with AJPBD and GBC without AJPBD. Results: Among the 14 patients with AJPBD, there were five fusiform, four c ystic, and two cylindric dilatations of the biliary tract and three nondila ted bile ducts. Nine (64%) of 14 patients had GBC, five with fusiform dilat ations, two with cylindric dilatations, and two with nondilated bile ducts. No patient with cystic dilatation had GBC. There were two stage I cancers, four stage II, two stage III, and one stage IVB. One patient with stage IV B GBC died of recurrence 8 months after operation. The remaining 8 patients were well without signs of recurrence from 8 to 72 months after operation. The frequency of grossly polypoid or histologically papillary adenocarcino ma was higher in GBC patients with AJPBD than in those without AJPBD (p < 0 .01). The proportion of disease-free survivors was greater among GBC patien ts with AJPBD than among those without AJPBD (p < 0.05). Conclusions: AJPBD with noncystic dilatation or without dilatation appears to be an important risk factor for GBC. In this limited series, patients wi th GBC accompanied by AJPBD have had relatively favorable outcomes. (J Am C ell Surg 1999;189:57-62. (C) 1999 by the American College of Surgeons).