TAKE-HOME POINTS
■ Cholangiocarcinoma is a rare disease but has an increased incidence in patients with underlying sclerosing cholangitis or choledochal cysts.
■ Perihilar cholangiocarcinoma, also called Klatskin's tumor, is the most common. Klatskin's tumors are classified with the Bismuth-Corlette system.
■ Surgical resection is the only curative treatment for cholangiocarcinoma. Resectability is largely determined by the extent of ductal and vascular involvement.
■ Patients have a better than 5-year survival rate when an R0 resection is obtained.
CASE
A 65-year-old white man presented to his primary care physician (PCP) complaining of pruritus, jaundice, and a 2-month history of dark urine. He also admitted to an unintentional 10-lb weight loss in 2 months, anorexia, and epigastric fullness after meals. He denied fever, sweats/chills, abdominal pain, nausea, vomiting, change in bowel or bladder habits, chest pain, shortness of breath, and headaches.
The patient's medical and surgical histories were unremarkable; he had no prior hospitalizations; and he had no significant family history. He denied alcohol, tobacco, and illicit drug use. His medications included oral ciprofloxacin, 750 mg three times a day, for 1 month to treat a biliary tract infection. The patient had no known drug allergies.
The PCP ordered CT, which revealed an ill-defined mass measuring 2.1 × 2.8 × 2.6 cm at the level of the right hepatic duct with intrahepatic ductal dilatation and encasement of the right portal vein immediately beyond the main portal vein bifurcation (Figure 1). The patient subsequently underwent endoscopic retrograde cholangiopancreatography (ERCP) with placement of a common bile duct stent. Following the stent placement, the patient's total bilirubin level normalized and his jaundice resolved. The patient was then referred for surgical evaluation.
Presurgical physical examination The patient was in no acute distress. Vitals signs were temperature, 98.8°F; pulse, 62 beats per minute; BP, 112/60 mm Hg; respirations, 16 breaths per minute. Jaundice of the skin was apparent. There was slight scleralicterus. His abdomen was soft, nondistended, and nontender to palpation. There was no abdominal organomegaly or guarding. No Murphy's sign was noted. Heart rate and rhythm were regular with audible S1 and S2. Lungs were clear to auscultation in all fields, bilaterally. All other physical examination findings were unremarkable.
Pertinent laboratory studies, including liver function enzymes, and an MRI/magnetic resonance cholangiopancreatography (MRCP) were ordered. Laboratory test results were total bilirubin, 6.8 μmol/L; AST, 157 U/L; ALT, 166 U/L; alkaline phosphatase, 271 U/L. MRI revealed a 1.9 × 1.6-cm hyperintense T2 lesion with delayed enhancement in the region of porta hepatis with intrahepatic ductal dilatation. Further cross-sectional imaging revealed encasement of the right portal vein. MRCP revealed a perihilar mass. Based on imaging studies, type IIIa Klatskin's tumor with ipsilateral right portal vein encasement was the suspected diagnosis.
Treatment The patient was admitted to the surgical service. Despite the biliary stent, the patient had persistent intra-hepatic ductal dilation and hyperbilirubinemia. Percutaneous transhepatic cholangiography (PTC) was performed, and a percutaneous catheter was placed to more adequately drain the biliary system and correct the patient's bilirubin level before surgery (Figure 2).
Cross-sectional imaging and the cholangiogram indicated that the tumor was resectable because it involved only the right hepatic biliary ducts (type IIIa); and although vascular extension of the tumor was present, it only involved the ipsilateral branch of the right portal vein. An extended right hemihepatectomy (removal of liver segments IV, V, VI, VII, and VIII) would be required to extirpate all the disease; therefore, preoperative liver volumetrics were obtained to ensure that the future liver remnant (segments I, II, and III) would be of adequate volume to avoid postoperative liver insufficiency. Volumetrics revealed that the remnant encompassed 34% of the entire liver volume and was deemed to be adequate. The patient was taken to the operating room after confirming normalization of the bilirubin level.
At surgery, it became evident that the tumor involved not only the biliary confluence and right hepatic ducts, but also part of the left hepatic duct. An extended right hemihepatectomy with en bloc resection of the extrahepatic biliary tree was performed. Dissection of the left hepatic duct was taken up to the base of the umbilical fissure (the margin was noted to be negative). In addition, the right portal vein was found to be completely involved with the tumor. Specifically, it was involved down to the level of the bifurcation of the main portal vein. Given this, the right portal vein and the main portal vein confluence were resected. The left portal vein was then anastomosed primarily to the main portal vein to reconstruct the portal vein anatomy.