Abstract
Simple SummaryLong-term survival for patients with perihilar cholangiocarcinoma (pCCA) is rare. The median overall survival of patients undergoing curative-intent surgery for pCCA is 19 to 39 months. This multicenter study aimed to determine the cure rate and to identify clinicopathological factors that may preclude cure. Four hundred and sixty patients were included with a median follow-up of 10 years. Median OS was 29.9 months. Twenty-nine (6%) patients reached 10-year OS. The observed cure rate was 5%. Factors that virtually precluded cure (i.e., below 1%) according to the mixture cure model included age above 70, Bismuth–Corlette type IV tumors, hepatic artery reconstruction, and positive resection margins. Cure was unlikely (i.e., below 3%) in patients with positive lymph nodes or poor tumor differentiation. These factors need to be considered in patient counseling and long-term follow-up after surgery.Complete resection of perihilar cholangiocarcinoma (pCCA) is the only potentially curative treatment. Long-term survival data is rare and prognostic analyses are hindered by the rarity of the disease. This study aimed to determine the cure rate and to identify clinicopathological factors that may preclude cure. All consecutive resections for pathologically confirmed pCCA between 2000 and 2009 in 22 centers worldwide were included in a retrospective cohort study. Each center included its retrospective data series. A total of 460 patients were included with a median follow-up of 10 years for patients alive at last follow-up. Median overall survival (OS) was 29.9 months and 10-year OS was 12.8%. Twenty-nine (6%) patients reached 10-year OS. The observed cure rate was 5%. Factors that virtually precluded cure (i.e., below 1%) according to the mixture cure model included age above 70, Bismuth-Corlette type IV tumors, hepatic artery reconstruction, and positive resection margins. Cure was unlikely (i.e., below 3%) in patients with positive lymph nodes or poor tumor differentiation. These factors need to be considered in patient counseling and long-term follow-up after surgery.
Highlights
Perihilar cholangiocarcinoma is a rare malignancy that manifests at or near the biliary confluence and accounts for 50–60% of all cholangiocarcinomas [1]
In a large international cohort, we found an observed cure rate (i.e., 10-year overall survival (OS) without evidence of recurrence at last follow-up) of 5% after resection of perihilar cholangiocarcinoma (pCCA)
Previous studies that aimed at predicting survival after resection have repeatedly identified prognostic factors such as resection margin, tumor differentiation, lymph node involvement, vascular infiltration, and presence of metastasis [16,17,18]
Summary
Perihilar cholangiocarcinoma (pCCA) is a rare malignancy that manifests at or near the biliary confluence and accounts for 50–60% of all cholangiocarcinomas [1]. Patients and clinicians are confronted with many challenges from initial presentation until the definitive treatment. Obstacles faced range from confirming malignancy to biliary drainage with the risk of subsequent cholangitis. The majority of patients are eventually not amenable to surgery because of locally advanced or metastatic disease at the time of presentation [3]. This leaves the possibility of resection reserved for less than half of all patients (33.0–41.3%) [2,3,4]
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