PMC5536823_01
A The findings of endoscopic retrograde cholangiography performed at the initial diagnosis are shown. Bile duct irregularities were observed in the region from the bilateral hepatic ducts to the peripheral bile duct, and a nodule measuring 1.5 cm in diameter was seen in the common hepatic duct (arrow). B: An endoscopic examination showed mucinous discharge from the ampulla of Vater. C: Peroral cholangioscopy showed papillary tumors (salmon roe-like lesions) located in the common hepatic duct.
gr1.jpg
PMC5536823_01_gr1.jpg
fig0005
In addition, the common bile duct was dilated (to 1.5 cm in diameter) and contained a nodule measuring 1 cm in diameter (Fig. 1A). In addition, many round tumors (salmon roe-like lesions) were observed in the region from the middle common bile duct to the common hepatic duct (Fig. 1B/C).
A/B/C Magnetic resonance cholangiopancreatography showed that the nodule in the common hepatic duct had grown from 1.5 cm to 3 cm. A: the initial diagnosis, B: 3 months later, C: 6 months later; D/E: Computed tomography (CT) showed that the nodule in the common hepatic duct had grown. D: the initial diagnosis, E: 6 months later; F/G: CT revealed that the irregularities in the region from the left hepatic duct to the umbilical portion had disappeared. F: the initial diagnosis, G: 6 months later.
gr2.jpg
PMC5536823_01_gr2.jpg
fig0010
MRCP and CT revealed that the nodule in the common hepatic duct had increased in size from 1.5 cm to 3 cm and that the hilar bile duct was obstructed (Fig. 2A/B/C/D/E). On the other hand, CT demonstrated that the irregularities extending from the left hepatic duct to the umbilical portion had disappeared (Fig. 2F/G).
A The macroscopic appearance of the resected extrahepatic bile duct is shown. A papillary tumor measuring 3.0 x 1.5 cm was observed in the region from the right hepatic duct to the middle common bile duct (arrow). CBD: common bile duct, LHD: left hepatic duct; B: A histological image of the papillary tumor is shown. Intraductal papillary adenocarcinoma in situ was observed in part of the tumor. C: A histological image of the stump of the left hepatic duct is shown. Intraductal papillary adenoma in situ was detected.
gr3.jpg
PMC5536823_01_gr3.jpg
fig0015
During a macroscopic examination, a papillary tumor measuring 3.0 x 1.5 cm was observed in the region extending from the right hepatic duct to the middle common bile duct (Fig. 3A). A histological examination showed that the tumor was mainly composed of intraductal papillary adenoma; however, some part of it was composed of intraductal papillary adenocarcinoma in situ (Fig. 3B/C).
A/B Magnetic resonance imaging revealed dilation of the B3 and B4 ducts (to 1 cm) and small papillary tumors in the intrahepatic bile duct (arrow). The dilation of the B2 duct was mild. C: Percutaneous transhepatic bile duct (PTBD) tubes were inserted from the B3 duct to the reconstructed jejunal limb through the hepaticojejunostomy and from the B3 duct to the B2 duct. D: The discharge from the PTBD tube inserted from the B3 duct to the reconstructed jejunal limb contained crystallized mucin, but not bile (left side), while the discharge from the PTBD tube inserted from the B3 to the B2 duct contained pure bile (right side). E: The observed changes in the serum total bilirubin (T-bil) level are shown. Cholangitis and jaundice occurred frequently.
gr4.jpg
PMC5536823_01_gr4.jpg
fig0020
MRI revealed dilation of the B3 and B4 ducts (to 1 cm), and small papillary tumors were detected in the intrahepatic bile duct (Fig. 4A/B). At 48 postoperative months, a PTBD tube was inserted from the B3 to B2 duct (Fig. 4C). The discharge collected from the PTBD tube inserted into the region extending from the B3 duct to the reconstructed jejunum limb contained crystallized mucin, but not bile, while the PTBD discharge collected from the region extending from the B3 duct to the B2 duct was composed of pure bile (amount: 200 ml daily) (Fig. 4D). The patient has survived for over 7 years since the initial treatment in spite of the frequent occurrence of cholangitis and jaundice, which was treated via careful management of his biliary tract (Fig. 4E).