A 54-year-old woman visited our hospital on January 30, 2021 with a 1-day history of pain in the middle upper abdomen. On the day before the admission, she experienced pain in the middle upper abdomen with no obvious precipitating factors. The pain was paroxysmal but not radiating and accompanied yellowing of the skin and eyes. There was no manifestation of fever, vomiting, or diarrhea. When she visited our emergency department, abdominal enhanced CT was performed, wherein dilation of the upper common bile duct (with soft tissue shadows) and intrahepatic bile duct was observed, which led us to suspect a tumor. The patient was then admitted due to an initial diagnosis of a bile duct tumor. Physical examination revealed mild yellowing of the skin and sclera, and no tenderness or palpable mass was noted in the abdomen. The patient reported a previous history of diabetes, which was being managed with oral metformin. She denied having any past medical history of hypertension, heart disease, hepatitis B, tuberculosis, renal disease, pulmonary disease, and smoking and alcohol abuse. She underwent myomectomy three years ago. Routine blood test findings were normal. Blood biochemical profile revealed the following: total bilirubin = 96.1 micromol/L (normal range: 2-25 micromol/L), direct bilirubin = 54.3 micromol/L (normal range: 0-10 micromol/L), indirect bilirubin = 41.8 micromol/L (normal range: 0-10 micromol/L) and alanine aminotransferase (ALT) 616.4 IU/L (normal range: 9-50 IU/L). Tumor markers, including carcinoembryonic antigen (CEA), cancer antigen 19-9 (CA19-9), and alpha-fetoprotein (AFP), were normal. Immunoglobulin G4 (IgG4) was normal as well.
On enhanced MRI of the upper abdomen, the bile duct of the hilum was involved with intrahepatic bile duct dilation, suggesting cholangiocarcinoma. Figure 1 presents the T1 image and Figure 2 presents the T2 image, and the location of the swelling is indicated with a red arrow. In addition, the presence of multiple hepatic masses was noted as well. A small nodule was observed in the right lung. The hepatic mass was considered to possibly have metastatic potential, and its resection was planned. Multiple cysts were observed in both kidneys. Chest CT showed an atelectatic right lung and a ground-glass nodule in the right lower lobe. An initial diagnosis of cholangiocarcinoma of the hilum was made. The patient was then treated with laparoscopic resection of the bile duct tumor under general anesthesia + bile duct plastic repair + Roux-en-Y biliary-enteric anastomosis + resection of hepatic hemangioma. Cholestasis, evident gallbladder swelling, and common bile duct dilation (~2 cm) were identified during the procedure. In the meantime, two hyperechogenic small nodules (~0.8 cm) were identified in the liver VIII segment using an ultrasonic probe; these were partially resected using an ultrasonic knife and then pathologically diagnosed as hepatic hemangioma. The bile duct tumor of the hilum was resected along with the gallbladder. The duodenum of the common bile duct was detached, and the lower bile duct was ligated. The hepatoduodenal ligament was dissected. The bile duct above the bifurcation of the right and left hepatic ducts and the two bile ducts of the caudate liver lobes were detached. The separated bile ducts were cut. The tumor was located in the hilum and presented with yellow fat with an intact envelope (Figure 3). Rapid intraoperative pathology indicated negative bile duct margins, suggesting IMT. A Roux-en-Y biliary-enteric anastomosis was performed instead of right hemihepatectomy. First, the four separated bile duct openings were intermittently stitched together with 5-0 prolene sutures (Figure 4). Two silicone tubes were placed into the two bile duct openings of the caudate liver lobe, which supported smooth drainage and prevented the closure of the bile ducts. Bile duct plastic repair was performed laparoscopically as it offers low risk of biliary leakage after sutures; this was followed by the Roux-en-Y biliary-enteric anastomosis. Postoperative pathology identified the hepatic nodule as a hemangioma and revealed that the hilar mass presented with storiform hyperplasia of fibroblasts with interstitial collagen and infiltrating plasma cells and lymphocytes, which are consistent with the presentation of IMT (Figure 5). Besides, margin-negative tests were obtained in all bile ducts. Our surgery achieved R0 resection. Immunohistochemistry revealed smooth muscle actin (weak +), CD34 (-), CD117 (-), Des (-), ki67 (+) <5%, CD21 (-), and CD23 (-). Figure 6 shows positive staining for smooth muscle actin, and Figure 7 shows positive staining for Ki67 in tumor cells. Biliary IMT of the hilum was finally diagnosed. The patient was uneventful after the surgery and had no biliary leakage, abdominal hemorrhage, or infection. The patient was discharged on day 7 postoperatively. No tumor recurrence or metastasis was noted at 1-year follow-up. The timeline of the patient's symptoms, treatment, and prognosis in our case is shown in Figure 8.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee(s) and were in compliance with the tenets of the Declaration of Helsinki (as revised in 2013). Written informed consent was obtained from the patient for performing the procedures and publishing the findings as images and text.