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 of Oddi. A high incidence of gallbladder carcinoma (GBC) has been reported in adult patients with AJPBD.

STUDY DESIGN

Fourteen adult patients with AJPBD and 50 with GBC not associated with AJPBD were reviewed retrospectively to identify the clinical characteristics of AJPBD accompanied by GBC and to clarify the differences in clinicopathologic features between GBC associated with AJPBD and GBC without AJPBD.

RESULTS

Among the 14 patients with AJPBD, there were five fusiform, four cystic, and two cylindric dilatations of the biliary tract and three nondilated bile ducts. Nine (64%) of 14 patients had GBC, five with fusiform dilatations, 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 IVB 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 adenocarcinoma 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 patients 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 with GBC accompanied by AJPBD have had relatively favorable outcomes.