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Background: Effective diagnosis and treatment of patients with hilar cholangiocarcinoma (HCCA) is based on the synergy of endoscopists, interventional radiologists, radiotherapists and surgeons. This report summarizes the multidisciplinary experience in management of HCCA over a period of two decades at the Academic Medical Center in Amsterdam, with emphasis on surgical outcome., Methods: From 1988 until 2003, 117 consecutive patients underwent resection on the suspicion of HCCA. Preoperative work-up included staging laparoscopy, preoperative biliary drainage, assessment of volume/function of future remnant liver and radiation therapy to prevent seeding metastases. More aggressive surgical approach combining hilar resection with extended liver resection was applied as of 1998. Outcomes of resection including actuarial 5-year survival were assessed., Results: Eighteen patients (15.3%) appeared to have a benign lesion on microscopical examination of the specimen, leaving 99 patients with histologically proven HCCA. These 99 patients were analysed according to three 5-year time periods of resection, i.e. period 1 (1988-1993, n=45), 2 (1993-1998, n=25) and 3 (1998-2003, n=29). The rate of R0 resections increased and actuarial five-year survival significantly improved from 20±5% for the periods 1 and 2, to 33±9% in period 3 (p<0.05). Postoperative morbidity and mortality in the last period were 68% and 10%, respectively., Conclusion: Extended surgical resection resulted in increased rate of R0 resections and significantly improved survival. Candidates for resection should be considered by a specialized, multidisciplinary team., (Copyright © 2010 Elsevier Ltd. All rights reserved.)

Background: Surgical treatment of hilar cholangiocarcinoma (Klatskin tumours) is difficult because of its central location in the liver hilum. Recent developments in surgical techniques have improved the outcome after resection., Aim: To describe the surgical approaches currently applied in our centre and the impact of these strategies on outcome and criteria for resection., Methods: From 1988 to 2003, 99 consecutive patients underwent resection for hilar cholangiocarcinoma. Patients were analysed for rate of R0 resections in relation with Bismuth classification. Morbidity, mortality and survival were assessed., Results: The rate of hilar resections in combination with (extended) liver resections for type III and IV tumours increased from 24% to 95% in the last 5 years of the study period. Eight patients (8%) had Bismuth type IV tumours. Four of these patients underwent palliative local excisions of the hepatic duct confluence whereas the other four patients underwent hilar resection in combination with partial liver resection, resulting in microscopically radical resections. There was no mortality in this group. Overall postoperative morbidity and mortality were 68% and 10%, respectively., Conclusions: An aggressive surgical approach consisting of hilar resections combined with partial liver resections including segments 1 and 4, resulted in a higher rate of R0 resections. Even Bismuth type IV tumours may be resectable depending on the biliary anatomy of the hepatic duct confluence.

Background: Treatment of hilar cholangiocarcinoma (Klatskin tumors) has changed in many aspects. A more extensive surgical approach, as proposed by Japanese surgeons, has been applied in our center over the last 5 years; it combines hilar resection with partial hepatectomy for most tumors. The aim of this study was to assess the outcome of a 15-year evolution in the surgical treatment of Klatskin tumors., Methods: A total of 99 consecutive patients underwent resection for hilar cholangiocarcinoma in three 5-year time periods: periods 1 (1988-1993; n=45), 2 (1993-1998; n=25), and 3 (1998-2003; n=29). Outcome was evaluated by assessment of completeness of resection, postoperative morbidity and mortality, and survival., Results: The proportion of margin negative resections increased significantly from 13% in period 1 to 59% in period 3 (P<.05). Two-year survival increased significantly from 33%+/-7% and 39%+/-10% in periods 1 and 2 to 60%+/-11% in period 3 (P<.05). Postoperative morbidity and mortality were considerable but did not increase with this changed surgical strategy (68% and 10%, respectively, in period 3). Lymph node metastasis was, next to period of resection, also associated with survival in univariate analysis., Conclusions: Mainly in the last 5-year period (1998-2003), when the Japanese surgical approach was followed, more hilar resections were combined with partial liver resections that included segments 1 and 4, thus leading to more R0 resections. This, together with a decrease in lymph node metastases, resulted in improved survival without significantly affecting postoperative morbidity or mortality.

Objective: To find out how patients fared after palliative endoscopic biliary drainage for inoperable hilar cholangiocarcinoma., Design: Retrospective study., Setting: University hospital, the Netherlands., Subjects: Between 1992 and 1999, 41 patients who were referred for resection had tumours that were considered unresectable after additional investigations, including an exploratory laparotomy in 16 patients. In all patients, biliary drainage was established by endoscopic retrograde cholangiography (ERCP) and insertion of endoprostheses. Twelve patients also had percutaneous transhepatic biliary drainage (PTBD)., Results: The patients who did not have an exploratory laparotomy had fewer complications (1/25) than those who had explorations (4/16). All patients in both groups had one or more long-term complications during follow-up, of which cholangitis, jaundice, and abdominal pain were the most often recorded. In 32 patients, endoprostheses had to be replaced, a mean of 4 times. Median survival was 9 months, with no significant difference between the groups (8 and 11 months). Adjuvant radiotherapy had no influence on survival., Conclusion: The patients in this series had relatively long survival times, during which they had a substantial number of complications predominantly related to biliary drainage. Because biliary-enteric bypass operations result in effective relief of symptoms and excellent palliation, we suggest that when an exploration is done for patients with type III and IV tumours, a bypass should be made.

Background: The differentiation between benign and malignant strictures at the hepatic hilum is difficult. The aim of this study was to assess the clinical and radiographical features of hilar lesions in a series of patients resected for a presumed cholangiocarcinoma., Methods: Of 132 consecutive patients undergoing resection for a suspicious lesion at the hepatic hilum, 20 (15 per cent) had a histopathologically proven benign tumour, diagnosed as chronic fibrosing or erosive inflammation, sclerosing cholangitis, or a granular cell tumour. All medical files and radiological examinations of the patients were reassessed., Results: Clinical symptoms were considered suspicious in 16 of 19 patients. At ultrasonography only one of 16 patients had an unsuspicious examination. In 14 of 18 patients cholangiographic images were considered suspicious. Overall reassessment confirmed a suspicious lesion in all 20 patients., Conclusion: Because of the limitations of current diagnostic tools, a false-positive preoperative diagnosis of malignancy resulted in a 15 per cent resection rate of benign lesions in this series of suspicious hilar strictures.

The article explores the role of immunoglobulin G4-related sclerosing cholangitis in patients resected for presumed hilar cholangiocarcinoma (HCCA). Results reveal that features of autoimmune-like bile duct diseases were seen in nearly half of patients with benign hilar strictures resected for presumed HCCA. Frank immunoglobulin G4-related sclerosing disease was found in just two of the 15 patients with autoimmune-like bile duct disease.

The prognosis of patients with an unresectable bile duct cancer is poor. In 60–70% of patients, cholangiocarcinoma is located in the hepatic duct bifurcation and known as Klatskin tumour. Surgical resection offers the only chance for 5-year survival, but less than 20% are surgical candidates. Patients with unresectable cholangiocarcinoma are treated with biliary drains, but commonly die of liver failure or cholangitis due to biliary obstruction within 6 to 12 months. Chemotherapy and/or radiotherapy have not been evaluated in randomized, controlled trials. Photodynamic therapy (PDT) is a new and promising locoregional treatment, the aim of which is to destroy tumour cells selectively. PDT involves the injection of a photosensitizer followed by percutaneous or endoscopic direct illumination of the tumour with light of a specific wavelength. In recent non-randomized studies of small numbers of patients with unresectable cholangiocarcinoma, PDT induced a decrease in serum bilirubin levels, improved quality of life and a slightly better survival. Other non-randomized trials failed to show clinical benefits. Recently, the first prospective, randomized controlled study with PDT in a selected group of non-resectable cholangiocarcinoma patients was stopped prematurely. The improvement in survival in the PDT-randomized patients was so impressive that it was considered to be unethical to continue randomization. However, further studies are awaited in unselected patients with unresectable cholangiocarcinoma before PDT can be considered as the standard adjuvant therapy. [ABSTRACT FROM AUTHOR]

The main question for staging is resectability, which is reliant on vascular, longitudinal, and metastatic spread. Today, accurate staging of perihilar tumors is achieved by non-invasive diagnostic investigations. Direct cholangiography has been the gold standard as a diagnostic procedure in recent decades. Endoscopic retrograde cholangiopancreaticography (ERCP) often only shows the ducts below the obstruction, and visualization of an obstructed part of the biliary tree is often not possible. Direct cholangiography reveals no information about local tumor extension, lymph nodes, or vascular involvement. Because of the given limitations, potential complications (cholangitis, sepsis) associated with direct cholangiography and reduction of the accuracy of subsequent cross-sectional imaging studies, these invasive techniques should only be used in the case of palliative interventions. Endoscopic ultrasonography-guided fine-needle aspiration (EUS-FNA) can be used to assess the nature of biliary strictures and to derive information about the extent of periductal disease and the presence of lymph node metastases. In a study by Fritscher-Ravens, 44 patients with hilar strictures underwent EUS-FNA. The overall diagnostic accuracy, sensitivity, specificity, positive and negative predictive values were 91% (95% CI, 78.4-96.3%), 89% (95% CI, 73.3-96.8%), 100% (95% CI, 63.1-100%), 100% (95% CI, 88.8-100%), and 67% (95% CI, 34.9-90%), respectively. The planned surgical approach was changed in 27 of 44 patients. In 15-20% of cholangiocarcinoma, patients with unremarkable abdominal imaging studies have metastatic lymph node involvement according to EUS evaluation. Due to the risk of peritoneal seeding, however, EUS with FNA is not recommended in patients still with a potential curative tumor. [ABSTRACT FROM AUTHOR]