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

Abstract

AimPerihilar 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.MethodsWe 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 conclusionUpcoming 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.

Effect of structured use of preoperative portal vein embolization on outcomes after liver resection of perihilar cholangiocarcinoma

“…Data were obtained retrospectively from a maintained database. Patient management and work‐up have been described in detail previously. HBS is the current modality of choice for remnant liver assessment, and liver volumes are determined only when there is a discrepancy in the results obtained by HBS or at the surgeon's request.…”

A Molecular Diagnosis of Cholangiocarcinoma suggested using Cell-free DNA following Multiple Unsuccessful Attempts at Obtaining a Tissue Diagnosis.

“…percutaneous cholangioscopy or the spyglass used in the case described), florescence in situ hydridization (FISH) analysis of the cytology cell blocks of specimens obtained and repeated brushings or use of stiffer bristles in obtaining tissue. A worldwide study of resection for "presumed" cholangiocarcinoma underscores the challenge as 8-22% of patients "turned out to have benign disease on microscopic examination of the resection specimens [3]. " cfDNA NGS testing is increasingly used to identify actionable molecular alterations in DNA shed from tumor cells, as therapies aimed at counteracting the consequences of specific alterations may offer effective therapeutic options for these patients.…”

Preoperative Management of Patients Undergoing Liver Resection for Perihilar Cholangiocarcinoma

“…Nimura and colleagues introduced in the early '90s the concept of routine segment 1 resection for surgical treatment of PHC (38), demonstrating an improvement in the 5-year survival rate (40% vs 5%) (38,39). Extended hepatectomies are nowadays considered the standard of treatment for PHC (40). A low-volume FLR is therefore often anticipated in these patients and a preoperative angio-CT for volumetric evaluation has to be part of the preoperative work-up for PHC.…”

“…An uptake <2.69 %/min/m² of the FLR has been associated with the risk of PHLF and mortality after extended hepatectomy (52). Recently the Amsterdam Medical Centre has included HBS as part of the preoperative work-up for PHC (40). The authors believe that HBS could provide additional information on the liver quality, but results on the HSB impact on PHLF in patients with PHC are still awaited.…”

“…Currently, indications for PVE include a FLR <40% of the total liver volume (40,62) or low function of the FLR, quantified as an HBS function <2.7%/min/m 2 (40) or an ICG clearance of the FLR < 0.0548). Recently, a score based on FLR volume, bilirubin level and cholangitis has been developed to select patients for PVE (44).…”