Modern work-up and extended resection in perihilar cholangiocarcinoma: the AMC experience

Abstract

Aim: Perihilar cholangiocarcinoma (PHC) is a challenging disease and requires aggressive surgical treatment in order to achieve curation. The assessment and work-up of patients with presumed PHC is multidisciplinary, complex and requires extensive experience. The aim of this paper is to review current aspects of diagnosis, preoperative work-up and extended resection in patients with PHC from the perspective of our own institutional experience with this complex tumor.

Methods: We provided a review of applied modalities in the diagnosis and work-up of PHC according to current literature. All patients with presumed PHC in our center between 2000 and 2016 were identified and described. The types of resection, surgical techniques and outcomes were analyzed.

Results and conclusion: Upcoming diagnostic modalities such as Spyglass and combinations of serum biomarkers and molecular markers have potential to decrease the rate of misdiagnosis of benign, inflammatory disease. Assessment of liver function with hepatobiliary scintigraphy provides better information on the future remnant liver (FRL) than volume alone. The selective use of staging laparoscopy is advisable to avoid futile laparotomies. In patients requiring extended resection, selective preoperative biliary drainage is mandatory in cholangitis and when FRL is small (< 50%). Preoperative portal vein embolization (PVE) is used when FRL volume is less than 40% and optionally includes the left portal vein branches to segment 4. Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) as alternative to PVE is not recommended in PHC. N2 positive lymph nodes preclude long-term survival. The benefit of unconditional en bloc resection of the portal vein bifurcation is uncertain. Along these lines, an aggressive surgical approach encompassing extended liver resection including segment 1, regional lymphadenectomy and conditional portal venous resection translates into favorable long-term survival.

Keywords: Biliary drainage; Biomarkers; Diagnosis; Hepato-biliary scintigraphy; Klatskin tumor; Perihilar cholangiocarcinoma; Postoperative outcome; Preoperative assessment; Staging; Surgical resection.

Fig. 1

Bismuth-Corlette classification for staging of perihilar cholangiocarcinoma

Fig. 2

Flow diagram of patients referred to the AMC with suspicion on PHC (2000–2016)

Fig. 3

Overall survival in 170 patients undergoing resection of pathology proven PHC in the AMC. The 5-year survival rate after resection was 44.3%

Fig. 4

Flowchart showing work-up and treatment of patients suspected of PHC

Fig. 5

Extended resection for PHC should include the central sector (segment 4) with segment 1 along the antero-posterior axis of the liver. Depending on the predominant side of the tumor, a left (extended) or right extended hemihepatectomy is chosen for en bloc resection of the hilar area