Management Of Hilar Cholangiocarcinoma Research Articles

Liver transplantation for cholangiocarcinoma: Past, present, and the future

Management of hilar cholangiocarcinoma (CCA) remains a major challenge incorporating complex diagnostic and therapeutic modalities. The management has evolved over the decades in pursuit of optimal therapeutic outcome for this challenging pathology. The only curative therapeutic option for CCA is complete (R0) surgical resection with negative margins. Curative resection for hilar CCA (HCCA) remains a surgical challenge due to its high propensity for invasion into liver parenchyma, encasement of portal vessels, and metastasis to regional lymph nodes. Liver transplantation (LT) was proposed as an alternative therapeutic option, but the initial results were extremely disappointing due to high rate of recurrence. The management has evolved over decades with introduction of neoadjuvant treatment options followed by LT resulting in optimal outcomes for an otherwise lethal disease. The current review outlines the changing trends in the management of HCCA over the years.

Looking Forward: Surgical Resection, Liver Transplantation, and Hilar Cholangiocarcinoma

This is a review of recent clinical advances impacting the management of hilar cholangiocarcinoma. Hilar cholangiocarcinoma (CCA) is the second most common primary liver malignancy, with an incidence in the USA of approximately 1.2 in 100,000. At present, there are no effective medical therapies, and thus far, the only potentially curative options include surgical resection or neoadjuvant chemoradiotherapy followed by liver transplantation. Although surgical resection remains the standard of care, the majority of patients unfortunately present with unresectable disease. Hilar CCA arises in two subsets of patients: those with de novo disease and those with underlying inflammation, which in western countries is typically primary sclerosing cholangitis (PSC). For patients with early-stage unresectable de novo disease and for patients with hilar CCA arising in the setting of PSC, liver transplantation is the only surgical option and can achieve 65% 5-year survival. While surgical resection remains the standard approach for patients with resectable disease, determining resectability continues to be difficult, and careful consideration must be undertaken in this decision process. Recent changes in surgical technique, primarily the use of en bloc resection, have resulted in improved surgical outcomes. Because adjunct medical therapies remain limited, current work has aimed to identify prognostic factors influencing patient outcomes and to improve endoscopic detection of CCA through optimization of biliary fluorescence in situ hybridization (FISH). At present, surgical resection and liver transplantation continue to be the only effective treatment options for hilar CCA. Recent advances in treating hilar CCA have resulted in refinements of surgical technique, improvements in diagnostic testing, as well as the identification of prognostic factors impacting patient outcomes and survival.

Robotic radical resection for hilar cholangiocarcinoma: perioperative and long-term outcomes of an initial series.

Radical resection for hilar cholangiocarcinoma (HCa) is one of the most challenging abdominal procedures. Robotic-assisted approach is gaining popularity in hepatobiliary surgery but scarcely tried in the management of HCa. We herein report our initial experience of robotic radical resection for HCa. Between May 2009 and October 2012, 10 patients underwent fully robotic-assisted radical resection for HCa in a single institute. The perioperative and long-term outcomes were analyzed and compared with a contemporaneous 32 patients undergoing traditional open surgery. The 10 patients presented one of Bismuth-Corlette type II, four of IIIa, one of IIIb and four of IV. There was no significant clinicopathological disparity between the robotic and open groups. The robotic radical resection involves hemihepatectomy plus caudate lobectomy or trisectionectomy, extrahepatic bile duct resection, radical lymphadenectomy and Roux-en-Y hepaticojejunostomy. No conversion to laparotomy occurred. Robotic resection compared unfavorably to traditional open resection in operative time (703±62 vs. 475±121min, p<0.001) and morbidity [90 (9/10) vs. 50%, p=0.031]. No significant difference was found in blood loss, mortality and postoperative hospital stay. Major complications (≥Clavien-Dindo III) occurred in three patients of robotic group. One patient died of posthepatectomy liver failure on postoperative day 18. The hospital expenditure was much higher in robotic group (USD 27,427±21,316 vs. 15,282±5957, p=0.018). The tumor recurrence-free survival was inferior in robotic group (p=0.029). Fully robotic-assisted radical resection for HCa is technically achievable in experienced hands and should be limited to highly selective patients. Our current results do not support continued practice of robotic surgery for HCa, until significant technical and instrumental refinements are demonstrated.

Hilar Cholangiocarcinoma: expert consensus statement

An American Hepato-Pancreato-Biliary Association (AHPBA)-sponsored consensus meeting of expert panellists met on 15 January 2014 to review current evidence on the management of hilar cholangiocarcinoma in order to establish practice guidelines and to agree consensus statements. It was established that the treatment of patients with hilar cholangiocarcinoma requires a coordinated, multidisciplinary approach to optimize the chances for both durable survival and effective palliation. An adequate diagnostic and staging work-up includes high-quality cross-sectional imaging; however, pathologic confirmation is not required prior to resection or initiation of a liver transplant trimodal treatment protocol. The ideal treatment for suitable patients with resectable hilar malignancy is resection of the intra- and extrahepatic bile ducts, as well as resection of the involved ipsilateral liver. Preoperative biliary drainage is best achieved with percutaneous transhepatic approaches and may be indicated for patients with cholangitis, malnutrition or hepatic insufficiency. Portal vein embolization is a safe and effective strategy for increasing the future liver remnant (FLR) and is particularly useful for patients with an FLR of <30%. Selected patients with unresectable hilar cholangiocarcinoma should be evaluated for a standard trimodal protocol incorporating external beam and endoluminal radiation therapy, systemic chemotherapy and liver transplantation. Post-resection chemoradiation should be offered to patients who show high-risk features on surgical pathology. Chemoradiation is also recommended for patients with locally advanced, unresectable hilar cancers. For patients with locally recurrent or metastatic hilar cholangiocarcinoma, first-line chemotherapy with gemcitabine and cisplatin is recommended based on multiple Phase II trials and a large randomized controlled trial including a heterogeneous population of patients with biliary cancers.

High-dose-rate brachytherapy planning in palliative management of hilar cholangiocarcinoma: a case study

AbstractIntroduction:Cholangiocarcinoma (CCA) or klatskin’s tumour involves malignant tumours at the liver hilum’s biliary confluence. Incidence of CCA results in unresectable tumours that require appropriate therapy to improve quality of life. The liver is considered as the most frequent site of tumour recurrence. Promising results of long-term survival have been established with computed tomography-guided high-dose-rate brachytherapy.Materials and methods:Intraluminal brachytherapy (ILBT) is performed through the percutaneous transhapatic bile duct drain tube (PTBD). The passage of the brachytherapy guide tube through the bile duct is more complex compared with oesophageal/endobronchial application.Results/discussion:It results in a recoiled view of the tube in the abdominal region of the computed tomography (CT) scan. Owing to inherent artefacts induced by metal stents in CT scans, intersected view is possible between the ILBT guide tube and the intra-hepatic drain tube. It would mislead the planner to track wrong passage that could result in fatal error.Conclusion:In this case study, we contoured the ILBT guide tube by cross-verifying its position with a digitally reconstructed radiograph (DRR) before catheter tracking. Thus, it ensures precise simulation of source dwell positions, thereby avoiding high-dose delivery to nearby vital organs such as intestines, liver hilum and blood vessels.

Multidisciplinary team diagnosis and treatment of hilar cholangiocarcinoma

The management of hilar cholangiocarcinoma was challenging to the surgeons due to difficult operations and low resection rate. Recently, a new mode of multidisciplinary team (MDT) is applied to the diagnosis and treatment of hilar cholangiocarcinoma, which leads to significant changes and development of the diagnosis and treatment for hilar cholangiocarcinoma. In this article, the authors first introduced the recent application of MDT treatment for hilar cholangiocarcinoma on diagnostic methods, staging systems, evaluation for resectability, radical resection, application of liver transplantation and other systematic therapies. Considering the complexity of hilar cholangiocarcinoma, a surgery-centered MDT for hilar cholangiocarcinoma is important and should be promoted. Key words: Biliary neoplasms, hilar; Multidisciplinary team; Diagnosis; Therapy

Problems and countermeasures in surgical therapy of hilar cholangiocarcinoma

Surgical resection offers hilar cholangiocarcinoma patients the only chance for cure and long-term survival.Hepatectomy,extrahepatic bile duct resection,regional lymphadenectomy and hepatojejunostomy are the current treatment of choice for most patients with hilar cholangiocarcinoma.Because of the aggressive nature of hilar cholangiocarcinoma and the absence of effective adjuvant therapy,surgical therapy still remains a challenge to hepatopancreatobiliary surgeons even in the precise surgery era.Controversies exist over preoperative assessment and staging,the use of preoperative biliary drainage,preoperative portal vein embolization,the range of hepatic resection,portal vein resection and construction,hepatic arteriectomy and the range of lymphadenectomy.This article reviews the surgical management of hilar cholangiocarcinoma with a focus on these controversies. Key words: Cholangiocarcinoma, hilar; Surgical procedures, operative; Problems,countermeasures

Appropriate choice of surgical strategies in managing hilar cholangiocarcinoma

Hilar cholangiocarcinoma is a rare tumor with a poor prognosis.Due to advances in preoperative imaging and enhanced comprehension of tumor biological behavior,surgical management of hilar cholangiocarcinoma has evolved since its original description.Currently,it has been accepted that complete surgical resection provides the only possibility for cure or long-term survival,however,the surgical management of hilar cholangiocarcinoma is extremely challenging because of its anatomical location and vascular proximity.In order to achieve complete resection, several surgical approaches have been investigated and evaluated regarding major hepatic resection,lymph node dissection,vascular resection,extended resection and liver transplantation,however,there are still many disputations. Furthermore,many surgical technical difficulties exist in biliary reconstruction after resection owing to anatomical problems.Focusing on the disputes and problems mentioned above,we herein review and discuss surgical strategies in managing hilar cholangiocarcinoma. Key words: Cholangiocarcinoma, hilar; Hepatectomy; Surgical procedures, operative

Endoscopic management of hilar cholangiocarcinoma

Hilar cholangiocarcinoma has a poor prognosis and surgery remains the only curative option. However, few patients are diagnosed at a curable stage and palliative therapies are, therefore, mandatory. Endoscopy could have a useful role in the work-up of patients with hilar cholangiocarcinoma who are unsuitable for surgery. Endoscopic retrograde cholangiopancreatography provides an opportunity to collect specimens for cytological or histological diagnosis, yet is often nondiagnostic. Other techniques, including fluorescence in situ hybridization, confocal laser endomicroscopy and endoscopic ultrasonography, are now improving the accuracy of tissue diagnosis. This Review presents an overview of the diagnostic and therapeutic role of endoscopic procedures in the management of hilar cholangiocarcinoma. The use of such procedures in guiding the therapeutic management of patients with hilar cholangiocarcinoma is discussed, and the relative success of endoscopic stenting as the main palliative therapy for obstructive jaundice (a common complication of hilar cholangiocarcinoma) is described. The potential role of photodynamic therapy as a palliative treatment for patients with hilar cholangiocarcinoma is also outlined.

Controversial issues in surgical management of hilar cholangiocarcinoma

Hilar cholangiocarcinoma should be identified from other malignant tumors and/or benign disease involving the hilum which may present similar image features.Clinal stage need to be further studied. Radical resection should be recommended in multidisciplinary treatment of hilar cholangiocarcinoma. The patients who were received initial surgical drainage and/or local recurrence after initial surgical resection of hilar cholangiocarcinoma, should actively be given the opportunity of radical reoperation, with potential long-term survival. Extended surgical resection resulted in increased rate of R0 resections and significantly improved survival. However, it also significantly increased the risk of serious postoperative complications, which must be balanced between survival advantage and surgical risk. Key words: Hilar cholangiocarcinoma; Surgery; Multidisciplinary treatment; Prognosis

Management of hilar cholangiocarcinoma: review of a 25-year experience

Hilar cholangiocarcinoma has received great attention in the past 25 years because of its rising prevalence. However, the results of surgical treatment of hilar cholangiocar-cinoma are still unsatisfactory. A series of studies on cholangio-carcinoma, both clinical and experimental, were conducted in the Research Institute of Hepatobiliary Surgery of PLA. Detailed clinicopathological research revealed the characteristics of nerve involvement and metastasis of bile duct cancer, which may be responsible for the high recurrence after surgical removal. Hepa-titis C virus may be one of the causal factors of bile duct cancer in China. Clinically, of the 402 patients with hilar cholangiocarcinoma, 51. 5% of the resections were found to be R0. The 5-year survival rate was 33. 3% and the morality rate was 0.3%. For those high-risk patients with jaundice, when resec-tion of the hepatic artery was found to be necessary, an arterial-portal venous shunt was constructed with satisfactory results. However, the best approach to treat hilar cholangiocarcinoma still needs further investigation. Key words: Cholangiocarcinoma, hilar; Surgical procedures, operative; Therapy

Analysis of the placement of multiple metallic stents in the treatment of hilar cholangiocarcinoma

目的 探讨应用金属内支架治疗复杂型肝门部胆管癌,特别是侵犯肝内胆管的肝门部胆管癌的介入治疗方法 .方法 肝门部胆管癌45例,其中BismuthⅡ型12例、Ⅲa型17例、Ⅲb型10例、Ⅳ型6例.45例均先行经皮经肝胆管引流术后实施胆管内支架置入术,分别以T、Y、X型或其组合方式留置2或3枚内支架.结果 45例患者内支架96枚全部置入成功,治疗后胆红素[(312±249)μmol/L]与治疗前[(51±42)μmol/L]相比明显下降(t=4.21,P＜0.01),支架置入后30 d内病死率为2.2%(1/45).平均支架开通时间181.5 d(26～473 d),平均生存时间215.3 d(26～516 d).结论 对于复杂型肝门部胆管癌,胆管内支架治疗是一种有效改善患者生存质量的方法 .特别对病变侵犯肝内胆管的病例,应选择不同的穿刺路径及支架留置方法 ,尽可能解决更多肝段的胆管引流。

A Novel Endoscopic Approach to Brachytherapy in the Management of Hilar Cholangiocarcinoma

Traditionally, biliary brachytherapy sources are placed percutaneously via transhepatic drains or endoscopically via nasobiliary tubes (NBT). Another ERCP is needed for stent replacement after NBT removal. The aim of this study was to determine the feasibility and safety of endoscopic transpapillary insertion of irradiation sources through 10-Fr stents. Medical records of Mayo Clinic Rochester patients undergoing biliary brachytherapy for hilar cholangiocarcinoma (CCA) were reviewed. Patients were part of a treatment protocol with curative intent including external beam radiation therapy (4,500 cGy), radiation sensitization (5-FU), and low dose rate (LDR) brachytherapy (<3,000 cGy) followed by liver transplantation. The 10-Fr biliary stent placed across the malignant biliary stricture was directly cannulated using a radiopaque (192)Ir embedded ribbon within a 300-cm long, 5.1-Fr plastic sheath. After withdrawal of the endoscope, the external end of the brachytherapy catheter was rerouted transnasally and secured. Each patient was hospitalized in a shielded room up to 24 h after which the brachytherapy catheter was removed by hand. Between 1999 and 2004, 32 patients underwent biliary brachytherapy via endoscopically placed 10-Fr plastic stents (mean age 50.6 yr, 69% PSC, bilateral brachytherapy catheters 28.1%). The technical complication observed was immediate brachytherapy catheter displacement (7 of 32, 22%) managed by prompt brachytherapy catheter repositioning. LDR biliary brachytherapy administration via endoscopically placed biliary stents is technically feasible and appears reasonably safe in select patients with unresectable perihilar CCA. Unlike NBTs, stents can potentially be placed in bilateral ductal systems to accommodate dual brachytherapy catheters when indicated.

A Novel Endoscopic Approach to Brachytherapy in the Management of Hilar Cholangiocarcinoma

Background: Biliary brachytherapy is usually performed via a percutaneous transhepatic approach. Most endoscopic approaches involve removal of existing biliary stents and placement of a nasobiliary tube (NBT); the radioactive source is then passed through the NBT. Another ERCP is needed for stent replacement. We describe a novel technique for endoscopic insertion of the radiation source through previously placed 10Fr stents. Aims: To review the Mayo Clinic Rochester experience and complications of endoscopically guided brachytherapy (EBT) for hilar cholangiocarcinoma. Methods: Medical records of all Mayo Clinic Rochester patients undergoing EBT without the use of nasobiliary drains were reviewed. Most patients were part of a treatment protocol with curative intent including external beam radiation therapy (4500cGy), chemotherapy, low dose rate brachytherapy (< 3000cGy) with bolus 5FU followed by liver transplantation. The brachytherapy target area was pre-determined using CT simulation. 10Fr biliary stents placed across the malignant biliary stricture were cannulated in freehand fashion using 192Ir embedded wires within a 300 cm long, 5.5Fr plastic sheath (Best industries, Springfield, Va). The wires were positioned fluoroscopically across the known stricture. After withdrawal of the endoscope, the proximal end of the wire was rerouted through the oropharynx out the nares. The patient was hospitalized in a lead-lined room for 24 hours after which the 192Ir wires were removed non-endoscopically. Results: Between 1998 and 2004, 34 patients underwent EBT for hilar cholangiocarcinoma without the use of NBT (mean age = 50.6 years, 76% male, 79% white, 59% PSC). 10 Fr plastic stents (12 to 15 cm lengths) were used. Unilateral 192Ir wires were placed in 23; bilateral wires were used in 11. Minor complications occurred in 15 patients (44%) and included radiation duodenitis (7), duodenal stricture (1), post procedure pancreatitis (1), early stent migration (2), early stent occlusion (1), pyloric channel or duodenal ulcer (3), and early cholangitis (2) - all managed non-surgically. Conclusions: Endoscopically guided biliary brachytherapy is technically feasible, safe and can be performed without the use of nasobiliary tubes. Stents remain in place to ensure biliary drainage. This unique method avoids the need for percutaneous procedures and may reduce the number of endoscopic procedures as compared to the NBT-guided method.

Management of hilar cholangiocarcinoma in the North of England: Pathology, treatment, and outcome

To assess the management and outcome of hilar cholangiocarcinoma (Klatskin tumor) in a single tertiary referral center. The notes of all patients with a diagnosis of hilar cholangiocarcinoma referred to our unit for over an 8-year period were identified and retrospectively reviewed. Presentation, management and outcome were assessed. Seventy-five patients were identified. The median age was 64 years (range 34-84 years). Male to female ratio was 1:1. Eighty-nine percent of patients presented with jaundice. Most patients referred were under Bismuth classification 3a, 3b or 4. Seventy patients required biliary drainage, 65 patients required 152 percutaneous drainage procedures, and 25 had other complications. Forty-one patients had 51 endoscopic drainage procedures performed (15 failed). Of these, 36 subsequently required percutaneous drainage. The median number of drainage procedures for all patients was three, 18 patients underwent resection (24%), nine had major complications and three died post-operatively. The 5-year survival rate was 4.2% for all patients, 21% for resected patients and 0% for those who did not undergo resection (P = 0.0021). The median number of admissions after diagnosis in resected patients was two and three in non-resected patients (P<0.05). Twelve patients had external-beam radiotherapy, seven brachytherapy, and eight chemotherapy. There was no significant benefit in terms of survival (P = 0.46) or hospital admissions. Resection increases survival but carries the risk of significant morbidity and mortality. Percutaneous biliary drainage is almost always necessary and endoscopic drainage should be avoided if possible.

Changing Strategies in Diagnosis and Management of Hilar Cholangiocarcinoma

Hilar cholangiocarcinoma is one of the most difficult tumors to stage and treat. This study aims to evaluate (1) the best diagnostic imaging, (2) the usefulness of preoperative biliary drainage, (3) the resectability rate, and (4) the results of palliative treatments and surgical resection. Seventy-six patients with hilar cholangiocarcinoma with a mean age of 64 ± 11 years were treated at our institution from 1989 to 1999. Patients were studied preoperatively using ultrasound, computed tomography (CT), and percutaneous cholangiography or magnetic resonance cholangiography. Forty-eight patients (63%) underwent palliative treatment. Twenty-eight patients underwent surgical curative therapy; 20 resections and 8 orthotopic liver transplantations (OLTs). Percutaneous transhepatic cholangiography was performed in 18 of 28 patients (64%), and magnetic resonance cholangiography in 5 patients; both methods were equally effective in establishing tumoral invasion of the biliary ducts. Five patients did not undergo either diagnostic modality. Excluding the patients who underwent OLT, no significant differences were found in surgical mortality (1 v 2 patients) or postoperative morbidity (100% v 66%) for patients with and without preoperative biliary drainage. The postoperative mortality rate was 11% (3 of 28 patients). The overall resectability rate was 37%. Mean survival in the surgical and palliative groups was 35 and 6 months, respectively (P < .0001). Patients who underwent OLT had a better 5-year survival rate than those treated by tumor resection (36% v 21%; P = .02). Combined chemotherapy and radiotherapy apparently did not provide a significant survival benefit. Helical CT and magnetic resonance cholangiography are useful techniques to delineate tumor extent and rule out vascular invasion and lymph node or liver metastases. No clear conclusions regarding preoperative drainage can be drawn from this study. A high resectability rate (37%) is feasible with major hepatectomy. (Liver Transpl 2000;6:786-794.)

Resection of hilar cholangiocarcinoma.

Objective: Morbidity and mortality involved in the resection of hilar cholangiocarcinoma were reviewed retrospectively. The clinicopathologic and laboratory parameters that might influence the patient's survival also were re-evaluated. Summary Background Data: Although much progress has been made in the diagnosis and management of hilar cholangiocarcinoma, long-term outlook for most patients remains poor. Surgical resection is usually prohibited because of its local invasiveness, and most patients can only be managed by palliative drainage. Recently, many surgeons have adopted a more aggressive resection with varying degrees of success. Several prognostic factors in bile duct carcinoma have been proposed; however, no reports have specifically focused on resected hilar cholangiocarcinoma and its prognostic survival factors using multivariate analysis. Methods: The clinical records and pathologic slides of 49 cases with resected hilar cholangiocarcinoma were reviewed retrospectively. Twenty clinical and laboratory parameters were evaluated for their correlation with postoperative morbidity and mortality, whereas 31 variables were evaluated for their significance with postoperative survival. Variables showing statistical significance in the first univariate analysis were included in the following multivariate analysis using stepwise logistic regression test for factors affecting morbidity and mortality and Cox stepwise proportional hazard model for factors influencing survival. Results: There were 5 in-hospital deaths, and the cumulative 5-year survival rate in 44 patients who survived was 14.9%, with a median survival of 14.0 months. Multivariate analysis disclosed that coexistent hepatolithiasis and lower serum asparate aminotransferase levels (90 U/L) had a significant low incidence of postoperative morbidity, whereas a serum albumin of less than 3 g/dL was the only significant factor affecting mortality. Regarding survival, univariate analysis identified eight significant factors: 1) total bilirubin 10 mg/dL, 2) curative resection, 3) histologic type, 4) perineural invasion, 5) liver invasion, 6) depth of cancer invasion, 7) positive proximal resected margin, and 8) positive surgical margin. However, multivariate analysis disclosed total bilirubin ≥ 10 mg/dL, curative resection, and histologic type as the three most significant independent variables. Conclusions: Surgical resection provides the best survival for bilar cholangiocarcinoma. An adequate nutritional support to increase serum albumin over 3g/dL is the most important factor to decrease postoperative mortality. Moreover, preoperative biliary drainage to decrease jaundice and a curative resection with adequate surgical margin are recommended if longer survival is anticipated. Patients with well differentiated adenocarcinoma seem to survive longer compared to those with moderately or poorly differentiated tumors.

Factors influencing postoperative morbidity, mortality, and survival after resection for hilar cholangiocarcinoma.

OBJECTIVE; Morbidity and mortality involved in the resection of hilar cholangiocarcinoma were reviewed retrospectively. The clinicopathologic and laboratory parameters that might influence the patient's survival also were re-evaluated. Although much progress has been made in the diagnosis and management of hilar cholangiocarcinoma, long-term outlook for most patients remains poor. Surgical resection is usually prohibited because of its local invasiveness, and most patients can only be managed by palliative drainage. Recently, many surgeons have adopted a more aggressive resection with varying degrees of success. Several prognostic factors in bile duct carcinoma have been proposed; however, no reports have specifically focused on resected hilar cholangiocarcinoma and its prognostic survival factors using multivariate analysis. The clinical records and pathologic slides of 49 cases with resected hilar cholangiocarcinoma were reviewed retrospectively. Twenty clinical and laboratory parameters were evaluated for their correlation with postoperative morbidity and mortality, whereas 31 variables were evaluated for their significance with postoperative survival. Variables showing statistical significance in the first univariate analysis were included in the following multivariate analysis using stepwise logistic regression test for factors affecting morbidity and mortality and Cox stepwise proportional hazard model for factors influencing survival. There were 5 in-hospital deaths, and the cumulative 5-year survival rate in 44 patients who survived was 14.9%, with a median survival of 14.0 months. Multivariate analysis disclosed that co-existent hepatolithiasis and lower serum asparate aminotransferase levels (<90 U/L) had a significant low incidence of postoperative morbidity, whereas a serum albumin of less than 3 g/dL was the only significant factor affecting mortality. Regarding survival, univariate analysis identified eight significant factors: 1) total bilirubin > or = 10 mg/dL, 2) curative resection, 3) histologic type, 4) perineural invasion, 5) liver invasion, 6) depth of cancer invasion, 7) positive proximal resected margin, and 8) positive surgical margin. However, multivariate analysis disclosed total bilirubin > or = 10 mg/dL, curative resection, and histologic type as the three most significant independent variables. Surgical resection provides the best survival for hilar cholangiocarcinoma. An adequate nutritional support to increase serum albumin over 3 g/dL is the most important factor to decrease postoperative mortality. Moreover, preoperative biliary drainage to decrease jaundice and a curative resection with adequate surgical margin are recommended if longer survival is anticipated. Patients with well-differentiated adenocarcinoma seem to survive longer compared to those with moderately or poorly differentiated tumors.