A huge intraductal papillary mucinous carcinoma of the bile duct treated by right trisectionectomy with caudate lobectomy

Won-Joon Sohn,Sungho Jo

doi:10.1186/1477-7819-7-93

Abstract

BackgroundBecause intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is believed to show a better clinical course than non-papillary biliary neoplasms, it is important to make a precise diagnosis and to perform complete surgical resection.Case presentationWe herein report a case of malignant IPMN-B treated by right trisectionectomy with caudate lobectomy and extrahepatic bile duct resection. Radiologic images showed marked dilatation of the left medial sectional bile duct (B4) resulting in a bulky cystic mass with multiple internal papillary projections. Duodenal endoscopic examination demonstrated very patulous ampullary orifice with mucin expulsion and endoscopic retrograde cholangiogram confirmed marked cystic dilatation of B4 with luminal filling defects. These findings suggested IPMN-B with malignancy potential. The functional volume of the left lateral section was estimated to be 45%. A planned extensive surgery was successfully performed. The remnant bile ducts were also dilated but had no macroscopic intraluminal tumorous lesion. The histopathological examination yielded the diagnosis of mucin-producing oncocytic intraductal papillary carcinoma of the bile duct with poorly differentiated carcinomas showing neuroendocrine differentiation. The tumor was 14.0 × 13.0 cm-sized and revealed no stromal invasiveness. Resection margins of the proximal bile duct and hepatic parenchyma were free of tumor cell. The patient showed no postoperative complication and was discharged on 10th postoperative date. He has been regularly followed at outpatient department with no evidence of recurrence.ConclusionConsidering a favorable prognosis of IPMN-B compared to non-papillary biliary neoplasms, this tumor can be a good indication for aggressive surgical resection regardless of its tumor size.

Highlights

Because intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is believed to show a better clinical course than non-papillary biliary neoplasms, it is important to make a precise diagnosis and to perform complete surgical resection.Case presentation: We report a case of malignant IPMN-B treated by right trisectionectomy with caudate lobectomy and extrahepatic bile duct resection
Considering a favorable prognosis of IPMN-B compared to non-papillary biliary neoplasms, this tumor can be a good indication for aggressive surgical resection regardless of its tumor size
IPMN-B have been accumulated since the first description of mucus producing papillary cholangiocarcinoma by Isogai et al, in 1986 [3], there are still controversies on several aspects of IPMN-B and its concept is in process of establishment

Summary

Background

Biliary intraductal neoplasms occur in both intrahepatic and extrahepatic bile ducts and are proposed to have two types; a flat and a papillary type [1,2]. Liver computed tomography (CT) and magnetic resonance imaging showed marked dilatation of the left medial sectional bile duct (B4) resulting in a bulky cystic mass with multiple internal papillary projections. This cystic mass was so huge as to displace the paren-. Duodenal endoscopic examination demonstrated a patulous ampullary orifice with mucin expulsion and endoscopic retrograde cholangiogram confirmed a marked aneurysmal dilatation of B4 with luminal filling defects (Figure 2A, 2B) These findings suggested IPMN-B with malignancy potential and prompted us to plan a curative extended major hepatectomy and extrahepatic bile duct resection.

Discussion

Conclusion