PMC4746402_01
Preoperative imaging. (a) An ultrasonography and (b) MRCP revealed extensive papillary tumor in LHD, CHD, and CBD (arrow). LHD: left hepatic duct, CHD: common hepatic duct, and CBD: common bile duct.
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The imaging, including ultrasound and magnetic resonance cholangiopancreatography (MRCP), demonstrated extensive intraductal tumors in the pancreas and biliary system, including the left hepatic duct, the anterior branch of right hepatic duct, the common bile duct down to the distal part, and the entire length of pancreatic duct (Figure 1).
Intraoperative findings. (a) Significant dilatation of extrahepatic bile duct (arrow) and mass-forming lesion at segment 4 (arrow head). (b) Operative field after removal of specimen. IVC: inferior vena cava, LGV: left gastric vein, and RHA: right hepatic artery.
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Regarding intraoperative findings, apart from the lesions depicted on preoperative imaging, there was a 2 cm mass-forming lesion at segment 4 of the liver (Figure 2(a)). We decided to perform left trisectionectomy, pyloric-preserving pancreaticoduodenectomy, and total pancreatectomy (Figure 2(b)).
The surgical specimen and a schematic illustration of histopathology grading. (a) The surgical specimen of combined left trisectionectomy, pancreaticoduodenectomy, and total pancreatectomy. The picture shows the opening along extrahepatic bile duct and cut surfaces of liver and pancreas that reveals numerous diffuse soft pink papillary growths occupying the whole extrahepatic bile duct (LHD, RHD, CHD, and CBD) and left intrahepatic segment ducts (B2-B4). The pancreatic head was accidently displaced; therefore, its pancreatic duct (PD) has been labeled by the use of arrow heads. The main PD was diffuse dilation with intraductal lesions prominent at the proximal part, and generalized atrophy of the pancreas parenchyma was noted. No lesion was seen in the gallbladder. (b) A schematic illustration of the surgical specimen histopathological grading showing areas with low-grade (white-dotted area), high-grade (shaded area), carcinoma in situ (black dot), and invasive carcinoma areas located beneath the schema (asterisk). Amp: ampulla of Vater, B2: bile duct of segment 2, B3: bile duct of segment 3, B4: bile duct of segment 4, CBD: common bile duct, CHD: common hepatic duct, LHD: left hepatic duct, PD: pancreatic duct, RHD: right hepatic duct, and S1: segment 1 of liver.
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Examination of specimens disclosed numerous intraductal papillary lesions present almost throughout the entire specimen (Figure 3). The IPNB was diffusely located in the dilated intrahepatic bile ducts of B2, B3, B4, and the extrahepatic bile duct, while the majority of the lesion was low-grade intraepithelium neoplasm (Figures 3(b) and 4(b)). High-grade lesions were noted on the left hepatic duct, medial segment duct, and B4 as well as the distal extrahepatic bile duct (Figure 3(b)). The invasive foci were found at B4 and distal bile duct (Figures 3(b) and 4(d)-4(f)), the invading tumor forming a 2 cm subcapsular mass at S4A (Figure 4(d)), and an infiltrative lesion located at the wall of the duodenum (Figures 4(e) and 4(f)), respectively. Low-grade intraepithelium neoplasm (IPMN) was presented in the pancreatic head as intraductal soft pink growths plus multiple indurated nodular lesions (Figures 3 and 4(c)).
Micrographs. (a-c) Intraductal papillary lesions: (a) intrahepatic bile duct: IPNB with carcinoma in situ; (b) common bile duct: low-grade IPNB; and (c) main pancreatic duct: low-grade IPMN. (d-f) Invasive lesions: (d) mucin-producing tubular adenocarcinoma from invasive IPNB lesion at S4 of liver; (e, f) invasive carcinoma from distal common bile duct; (e) invading carcinoma at the duodenal wall (DW) from the intraductal lesion of the distal common bile duct (IPNB), invasive tumor at duodenal wall (*), highlighted in (f) showing moderately differentiated tubular adenocarcinoma. Original magnification: (a-c) x100, (d, f) x200, and (e) x40.
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The histopathology revealed intraductal papillary neoplasm with gastric phenotype of bile duct and pancreas (Figure 4). The invasive foci were found at B4 and distal bile duct (Figures 3(b) and 4(d)-4(f)), the invading tumor forming a 2 cm subcapsular mass at S4A (Figure 4(d)), and an infiltrative lesion located at the wall of the duodenum (Figures 4(e) and 4(f)), respectively. Low-grade intraepithelium neoplasm (IPMN) was presented in the pancreatic head as intraductal soft pink growths plus multiple indurated nodular lesions (Figures 3 and 4(c)).