A 53-year-old man had been experiencing obstructive jaundice and significant weight loss for 1 month. He had been healthy until the jaundice occurred and was not taking a medication on a regular basis. No history of parasitic infestation was noted. Physical examination revealed no obvious finding except for slightly icteric sclera. Abnormal indicators of initial laboratory tests included total bilirubin at 2.3 mg/dL (0.3-1.5), direct bilirubin at 1.6 mg/dL (0-0.5), aspartate aminotransferase (AST)/alanine aminotransferase (ALT) at 122/131 IU/L (<36 IU/L), alkaline phosphatase (ALP) at 464 IU/L (42-121), and CA19-9 at 838.6 (0-37) IU/L.
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). Neither lymph node nor distant organ metastasis was detected. Therefore, clinical staging was T2N0M0. Given the patient's preferences, good performance status, and limitation of disease in the bile duct and pancreas, we planned to perform surgery.
The patient underwent right percutaneous transhepatic biliary drainage (PTBD) using an 8.5 Fr. self-retaining catheter. Ten days later, left portal vein embolization (PVE) was performed. A follow-up CT scan was conducted to assure adequacy of the liver remnant.
One month later, the operation was carried out. 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 postoperative course was uneventful, except for diabetes mellitus requiring insulin administration.
The surgical specimens consisted of the liver (segments 1-5 and 8), gallbladder, extrahepatic bile duct, duodenum, entire pancreas, and spleen. Examination of specimens disclosed numerous intraductal papillary lesions present almost throughout the entire specimen (Figure 3). The histopathology revealed intraductal papillary neoplasm with gastric phenotype of bile duct and pancreas (Figure 4). 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. Metastasis was found in lymph node along common hepatic artery (station 8). In the pancreas, the main pancreatic duct was dilatation, pronounced mostly at the distal part. 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)). The epithelium of the distal duct was denuded and the pancreas parenchyma presented atrophic changes. No high-grade lesions or invasive foci were seen in the pancreas.
The patient received Gemcitabine-Cisplatin chemotherapy due to lymph node involvement in the pathological result. A CT scan was performed 5 months after the operation, showing no tumor recurrence.
After the 4th cycle of chemotherapy, the patient developed cholangitis and was admitted at a provincial hospital. During admission, a CT scan was performed revealing multiple peritoneal metastases. In addition to the advancement of the disease, the patient's performance status was also poor. Therefore, the patient received only supportive treatment and eventually passed away. The overall survival time after surgery was 8 months.