Radiological staging of hilar cholangiocarcinoma]

Hilar cholangiocarcinoma is an adenocarcinoma arising from the bile duct epithelium, at the confluence of the right and left intrahepatic bile ducts, within the porta hepatis. In most cases, hilar cholangiocarcinomas are locally invasive tumors which have dismal prognosis if left untreated, with mean survival of approximately three months after the initial presentation. Recently, preoperative assessment of resectability and staging of hilar cholangiocarcinoma has gained importance in better management of the patients. Non-invasive cross-sectional radiological imaging has made considerable advances, and staging of hilar cholangiocarcinomas using radiological examination became more accurate in recent years. In this review, staging of hilar cholangiocarcinoma using computed tomography, magnetic resonance imaging, magnetic resonance cholangiopancreatography, and magnetic resonance angiography is described.     

Magnetic resonance imaging of cholangiocarcinoma

Cholangiocarcinoma arises from the bile ducts and is the most common primary malignancy of the biliary tree. Cholangiocarcinoma is classified according to its growth pattern: mass-forming, periductal-infiltrating, or intraductal-growing type. The majority of cholangiocarcinomas occur at the common hepatic duct (CHD) and its bifurcation, also referred to as Klatskin's tumor, but they also can occur in more peripheral branches within the hepatic parenchyma. Microscopically, cholangiocarcinoma represents an adenocarcinoma with a glandular appearance arising from the epithelium of the bile ducts. On magnetic resonance (MR) images, cholangiocarcinomas appear hypointense on T1-weighted images, and hyperintense on T2-weighted images. Central hypointensity can be seen on T2-weighted images and correspond to fibrosis. On dynamic MR images, cholangiocarcinomas show moderate peripheral enhancement followed by progressive and concentric filling in the tumor with contrast material. Pooling of contrast within the tumor on delayed MR images is suggestive of peripheral cholangiocarcinoma. The role of MR imaging in hilar cholangiocarcinoma is to confirm/reach a diagnosis and to assess resectability. Hilar cholangiocarcinoma shows the same signal intensity pattern of peripheral tumors both on T1- and T2-weighted images. On magnetic resonance cholangiopancreatography (MRCP) images, hilar cholangiocarcinoma appears as a moderately irregular thickening of the bile duct wall (>/=5 mm) with symmetric upstream dilation of the intrahepatic bile ducts. The aim of preoperative investigation in Klatskin tumors typically requires the evaluation of the level of biliary obstruction, the intrahepatic tumor spread, and the vascular involvement; it also needs to show any atrophy-hypertrophy complex. Because of its intrinsic high tissue contrast and multiplanar capability, MR imaging and MRCP are able to detect and preoperatively assess patients with cholangiocarcinoma, investigating all involved structures such as bile ducts, vessels and hepatic parenchyma. The main reason for surgical/imaging discrepancy is represented by the microscopic diffusion along the mucosa and in the perineural space.     

Hilar cholangiocarcinoma evaluated by three-dimensional CT cholangiography and rotating cine cholangiography

BACKGROUND/AIMS: To evaluate the usefulness of three-dimensional cholangiography and rotating cine cholangiography in depicting the anatomy of the hilar bile duct and tumor extension, and in planning surgical procedures for hilar cholangiocarcinomas. METHODOLOGY: Five patients with hilar cholangiocarcinoma and obstructive jaundice who underwent percutaneous transhepatic biliary drainage followed by resection were serially examined by cine cholangiography and three-dimensional cholangiography which were reconstructed from a helical computed tomography scan. Tumor extension to the bile ducts was prospectively diagnosed and the resection margin was planned using both cine and three-dimensional cholangiograms. The histological evaluation of the resected specimens were compared with preoperative findings of cholangiograms. RESULTS: The three-dimensional cholangiograms from vertical projection demonstrated the bile duct anatomy with excellent image quality. To assess tumor invasion to the intrahepatic bile ducts, cine cholangiograms from lateral and oblique projections were necessary. Selection of the surgical procedure was influenced by preoperative evaluations of the lesion on both three-dimensional and cine cholangiograms. Histologically, the resected margin was free from tumor in all cases. CONCLUSIONS: Three-dimensional and cine cholangiography allowed accurate assessment of the biliary system in patients with hilar cholangiocarcinoma, which was helpful for planning the surgical procedure.     

Duct-to-duct biliary reconstruction after radical resection of Bismuth Ⅲ a hilar cholangiocarcinoma

At present, radical resection remains the only effective treatment for patients with hilar cholangiocarcinoma. The surgical approach for R0 resection of hilar cholangiocarcinoma is complex and diverse, but for the biliary reconstruction after resection, almost all surgeons use Roux-en-Y hepaticojejunostomy. A viable alternative to Roux-en-Y reconstruction after radical resection of hilar cholangiocarcinoma has not yet been proposed. We report a case of performing duct-to-duct biliary reconstruction after radical resection of Bismuth Ⅲa hilar cholangiocarcinoma. End-to-end anastomosis between the left hepatic duct and the distal common bile duct was used for the biliary reconstruction, and a singlelayer continuous suture was performed along the bile duct using 5-0 prolene. The patient was discharged favorably without biliary fistula 2 wk later. Evidence for tumor recurrence was not found after an 18 mo follow- up. Performing bile duct end-to-end anastomosis in hilar cholangiocarcinoma can simplify the complex digestive tract reconstruction process.     

Hilar cholangiocarcinoma—surgical anatomy and curative resection

We have studied the surgical anatomy of the intrahepatic bile duct, hepatic hilus, and caudate lobe based on intraoperative findings and selective cholangiography of surgical patients and resected specimens, and have established the cholangiographic anatomy of the intrahepatic subsegmental bile duct. Thorough knowledge of the three-dimensional anatomy of the subsegmental bile duct, hepatic hilus, and caudate lobe is indispensable for curative surgery of hilar cholangiocarcinoma. We designed and actually performed 15 kinds of hepatic segmentectomies with caudate lobectomy and extrahepatic bile buct resection in 100 consecutive patients, with curative resection being possible in 82 patients. Postoperative survival after curative resection of hilar cholangiocarcinoma was better than expected, and the 5-year survival rates for all 82 patients with curative resection and for 55 patients with curative surgery without portal vein resection were 31% and 43%, respectively. Hepatic segmentectomy with caudate lobectomy and extrahepatic bile duct resection should be designed not only in accordance with the preoperative diagnosis of tumor extension into the intrahepatic bile ducts but also so that curative surgery for advanced hilar cholangiocarcinoma can be performed.     

Sclerosing Carcinoma of the Right Hepatic Duct at the Porta Hepatis: Anicteric Presentation of Early Hilar Cholangiocarcinoma

Asymptomatic presentation of hilar cholangiocarcinoma is exceptional. We report an asymptomatic patient presenting with an abnormal alkaline phosphatase who proved to have a unilateral hilar cholangiocarcinoma obstructing the right heptic duct at the porta hepatis. Hyperbilirubinemia was absent. Subsequent to partial tumor resection and palliative decompression with right intrahepatic cholangiojejunostomy, the patient developed cholangitis with abscess formation. The efficacy of biopsy, tumor resection, and surgical decompression in hilar cholangiocarcinoma is discussed. Postmortem histology revealed biliary cirrhosis and acute cholangitis in the obstructed right liver lobe and minimal portal fibrosis in the unobstructed left liver lobe. The absence of bile stasis suggests compensatory bile excretion mechanism in the unobstructed left lobe. We believe the clinical and pathological findings in the patient to be part of the early natural history of hilar cholangiocarcinoma. Complete workup of asymptomatic alkaline phosphatase elevations in patients without previous history of hepatobiliary disease may prove fruitful in revealing incipient localized, unilateral bifurcation tumors.     

Cholangiocarcinoma: modern advances in understanding a deadly old disease

Cholangiocarcinomas are tumors that arise anywhere in the biliary tract, presumably of cholangiocyte origin. The global incidence of this rare disease is on the rise. Several known risk factors exist, and link chronic biliary inflammation to the pathogenesis of cholangiocarcinoma. Among these, amplification of the epidermal growth factor receptor, the interleukin-6 signaling pathway, inducible nitric oxide, erb-2, and cyclooxygenase-2 are well defined. Most patients present late, with a median survival of months. Although, imaging studies and clinical context often indicate cholangiocarcinoma, pathologic and cytologic diagnosis is difficult to obtain. Advanced cytologic tests with fluorescence in situ hybridization or digital image analysis can increase diagnostic sensitivity. Surgical resection is the current therapy of choice for both intrahepatic and ductal cholangiocarcinomas. However, the 5-year survival is poor, with 60 to greater than 90% recurrence rates. In a single center experience, liver transplantation with neoadjuvant chemoirradiation, for highly selected patients, has a 5-year disease free survival of greater than 80%. Future targeted therapies will depend on a better understanding of the cellular and molecular biology of cholangiocarcinomas.     

Histological features of precancerous and early cancerous lesions of biliary tract carcinoma

Biliary tract carcinoma develops within the intrahepatic or extrahepatic biliary tree and gallbladder. Primary sclerosing cholangitis, hepatolithiasis, congenital choledochal cyst, liver fluke infection, pancreatobiliary maljunction, toxic exposures and hepatitis virus infection are risk factors for the development of human biliary carcinoma. The precise molecular abnormalities of biliary carcinogenesis are still unknown, but chronic inflammatory conditions induce the production of reactive oxygen or nitrogen species leading to DNA damage. Recent studies indicate that cholangiocarcinoma of the large bile duct may arise in premalignant lesions such as biliary intraepithelial neoplasm (BilIN) and intraductal papillary neoplasm of the bile duct (IPNB). BilIN and IPNB are generally confined to the large and septal‐sized bile duct. BilINs are occasionally observed in non‐biliary liver cirrhosis as well as chronic biliary disease. In contrast, the precursor lesion of intrahepatic cholangiocarcinoma of the small bile duct type remains unclear. We herein demonstrated the histological characteristics of different tumor development pathways from premalignant lesion to carcinoma in different sites of the biliary tree.     

Left hepatic trisegmentectomy for intraductal papillary cholangiocarcinoma: report of a case

We present a rare case of intraductal papillary cholangiocarcinoma in a 69 year-old man which was treated with left hepatic trisegmentectomy. The hepatic bile ducts were dilated by intraductal masses, which had extended into the intrahepatic bile ducts without involvement of the posterior inferior segmental duct (B6). The patient underwent left hepatic trisegmentectomy with hilar duct resection. The tumors in the posterior superior segmental duct (B7) were resected and biliary reconstruction was performed with a jejunal loop. Post-operative recovery was good, and the patient survived for 7 months after surgery.     

Endoscopic treatment of cholangiocarcinoma and carcinoma of the duodenal papilla by intraductal high-intensity US: Results of a pilot study

BACKGROUND: Local and infiltrative extension make some biliary carcinomas accessible to nonoperative intraductal destruction. This study assessed the clinical feasibility and short-term results of local tumor destruction with an intraductal high-intensity US probe during ERCP. METHODS: The probe is a flexible catheter with an 8 x 2.8 mm US transducer and a lumen for a guidewire. Ten patients (6 women, 4 men; mean age 74.8 years) were treated with this device. Lesions treated included carcinoma of the papilla (3), bile duct cholangiocarcinoma (2), Bismuth grade I and II hilar cholangiocarcinomas (4), and intrahepatic cystadenocarcinoma (1). Two patients underwent US therapy before surgery. Treatment was performed during standard ERCP: the probe was inserted through the malignant stricture and US therapy was applied over 360 degrees under fluoroscopic control. RESULTS: No serious adverse effects were observed; right upper abdominal pain developed in one patient for 12 hours. In one patient, histopathologic assessment of the resected tumor revealed extensive coagulation necrosis with inflammation up to 10 mm in depth surrounding the bile duct lumen. In the other operated patient, biopsy specimens from the treated portion of the bile duct were negative for malignancy. There was complete regression of cholangiocarcinoma of the bile duct in our patient, allowing for permanent stent removal (follow-up 30 months). A partial response was noted in 4 other patients and no response in 3 patients. CONCLUSIONS: This new method of intraductal tumor destruction by high-intensity US during ERCP is feasible and can induce objectively measurable tumor necrosis. Long-term follow-up will determine whether this method is curative in some cases and if it can reduce the need for biliary stent placement.     

Current surgical treatment for bile duct cancer

Since extrahepatic bile duct cancer is difficult to diagnose and to cure,a safe and radical surgical strategy is needed.In this review,the modes of infiltration and spread of extrahepatic bile duct cancer and surgical strategy are discussed.Extended hemihepatectomy,with or without pancreatoduodenectomy(PD),plus extrahepatic bile duct resection and regional lymphadenectomy has recently been recognized as the standard curative treatment for hilar bile duct cancer.On the other hand,PD is the choice of treatment for middle and distal bile duct cancer.Major hepatectomy concomitant with PD(hepatopancreatoduodenectomy)has been applied to selected patients with widespread tumors.Preoperative biliary drainage(BD)followed by portal vein embolization(PVE)enables major hepatectomy in patients with hilar bile duct cancer without mortality.BD should be performed considering the surgical procedure,especially,in patients with separated intrahepatic bile ducts caused by hilar bile duct cancer.Right or left trisectoriectomy are indicated according to the tumor spread and biliary anatomy.As a result,extended radical resection offers a chance for cure of hilar bile duct cancer with improved resectability,curability,and a 5-year survival rate of 40%.A 5-year survival rate has ranged from 24% to 39% after PD for middle and distal bile duct cancer.     

Surgery for cholangiocarcinoma

Surgical resection is the only potentially curative treatment for patients with cholangiocarcinoma. For both perihilar cholangiocarcinoma (pCCA) and intrahepatic cholangiocarcinoma (iCCA), 5‐year overall survival of about 30% has been reported in large series. This review addresses several challenges in surgical management of cholangiocarcinoma. The first challenge is diagnosis: a biopsy is typically avoided because of the risk of seeding metastases and the low yield of a brush of the bile duct. However, about 15% of patients with suspected pCCA are found to have a benign diagnosis after resection. The second challenge is staging; even with the best preoperative imaging, a substantial percentage of patients has occult metastatic disease detected at staging laparoscopy or early recurrence after resection. The third challenge is an adequate volume and function of the future liver remnant, which may require preoperative biliary drainage and portal vein embolization. The fourth challenge is a complete resection: a positive bile duct margin is not uncommon because the microscopic biliary extent of disease may be more extensive than perceived on imaging. The fifth challenge is the high post‐operative mortality that has decreased in very high volume Asian centres, but remains about 10% in many Western referral centres. The sixth challenge is that even after a complete resection most patients develop recurrent disease. Recent randomized controlled trials found conflicting results regarding the benefit of adjuvant chemotherapy. The final challenge is to determine which patients with cholangiocarcinoma should undergo liver transplantation rather than resection.     

Radical surgery of left-sided klatskin tumors

Left-sided cholangiocarcinoma includes hilar cholangiocarcinoma (HC), predominantly involving the left hepatic duct, and intrahepatic cholangiocarcinoma (ICC) in the left liver. Left hepatectomy, or left hepatic trisectionectomy, is indicated as radical surgery of left-sided HC or ICC with or without hilar bile duct invasion. Left lateral sectionectomy, or left medial sectionectomy, is performed for the small mass-forming type ICC. Left hepatic trisectionectomy is indicated for left-sided HC with further cancer progress along the right anterior sectional duct or left-sided ICC involving the right anterior section over the middle hepatic vein and/or the right anterior pedicle. Combined caudate lobe and extrahepatic bile duct resection are mandatory in cases of HC or ICC involving the hepatic confluence. Preoperative biliary drainage should be performed not only for jaundiced patients but also for non-icteric patients with right-sided biliary dilatation of the future remnant liver. Preoperative left trisegment portal vein embolization after biliary drainage of the right posterior section should be carried out prior to left hepatic trisectionectomy. Left hepatectomy has been used as a radical and safer surgical procedure, but in European countries has still been associated with higher morbidity and about 10% operative mortality. Japanese surgeons have had no hospital deaths after carrying out left hepatic trisectionectomy done after preoperative biliary drainage followed by left trisegment portal vein embolization to increase safety and to prolong postoperative survival for patients with locally advanced left-sided cholangiocarcinoma.     

Aggressive surgical treatment of hilar cholangiocarcinoma

Recent progress in surgical techniques for and the perioperative management of hilar cholangiocarcinoma has led to improved outcomes for aggressive liver and bile duct resections, which, however, still show considerable morbidity and mortality. In this article, the results of pioneers' attempts in hepatobiliary surgery for difficult hilar cholangiocarcinomas are reviewed. It is recommended that curative hepatobiliary resection should be performed for hilar cholangiocarcinoma, with careful preoperative management of patients complicated with several difficult conditions.     

Cholangiocarcinoma

Cholangiocarcinomas are rare malignant tumors composed of cells that resemble those of the biliary tract. On the basis of their anatomic location, cholangiocarcinomas can be classified as intrahepatic, extrahepatic and hilar tumors. For reasons that are not clear, the incidence of cholangiocarcinoma is increasing globally. Established risk factors, including conditions associated with chronic biliary tract inflammation, account for a small proportion of cases. Additional risk factors such as cirrhosis, infection with hepatitis B virus and hepatitis C virus are now becoming recognized. The diagnosis of cholangiocarcinoma requires the integration of clinical information, imaging studies of the hepatic parenchyma and biliary tract, tumor markers, and histology. In terms of the treatment options for cholangiocarcinoma, surgery can be curative, although few patients are candidates for surgery. Palliative biliary decompression can provide symptomatic relief. Advances in MRI and positron-emission tomography scanning, identification of new tumor markers, improved utility of biliary cytology, and the use of photodynamic therapy for adjunct treatment are all expected to enhance the diagnosis, evaluation and management of cholangiocarcinoma.     

Anatomical,histomorphological and molecular classification of cholangiocarcinoma

Cholangiocarcinoma constitutes a heterogeneous group of malignancies that can emerge at any point of the biliary tree. Cholangiocarcinoma is classified into intrahepatic, perihilar and distal based on its anatomical location. Histologically, conventional perihilar/distal cholangiocarcinomas are mucin‐producing adenocarcinomas or papillary tumours; intrahepatic cholangiocarcinomas are more heterogeneous and can be sub‐classified according to the level or size of the displayed bile duct. Cholangiocarcinoma develops through multistep carcinogenesis and is preceded by dysplastic and in situ lesions. Definition and clinical significance of precursor lesions, including biliary intraepithelial neoplasia, intraductal papillary neoplasms of the bile duct, intraductal tubulopapillary neoplasms and mucinous cystic neoplasm, are discussed in this review. A main challenge in diagnosing cholangiocarcinoma is the fact that tumour tissue for histological examination is difficult to obtain. Thus, a major clinical obstacle is the establishment of the correct diagnosis at a tumour stage that is amenable to surgery which still represents the only curable therapeutic option. Current standards, methodology and criteria for diagnosis are discussed. Cholangiocarcinoma represents a heterogeneous tumour with regard to molecular alterations. In intrahepatic subtype, mainly two distinctive morpho‐molecular groups can currently be discriminated. Large‐duct type intrahepatic cholangiocarcinoma shows a high mutation frequency of oncogenes and tumour suppressor genes, such as KRAS and TP53 while Isocitrate Dehydrogenase 1/2 mutations and Fibroblast Growth Factor Receptor 2‐fusions are typically seen in small‐duct type tumours. It is most important to ensure the separation of the given anatomical subtypes and to search for distinct subgroups within the subtypes on a molecular and morphological basis.     

Clinical clues to suspicion of IgG4-associated sclerosing cholangitis disguised as primary sclerosing cholangitis or hilar cholangiocarcinoma

Background and Aim: This study aimed to determine the clinical characteristics of immunoglobulin G4 (IgG4)‐associated sclerosing cholangitis (ISC) and provide clinical clues differentiating ISC from primary sclerosing cholangitis (PSC) or hilar cholangiocarcinoma (CCC). Methods: Sixteen patients with ISC manifesting as hilar/intrahepatic strictures were analyzed for clinical characteristics and compared with patients with PSC and hilar CCC as disease controls for histology and serum IgG4 levels. Results: Distinguished biliary imaging findings of ISC included multifocal biliary tree involvement (n = 14), concentric bile duct thickening with preserved luminal patency (n = 13), and relatively mild proximal dilatation, despite prominent bile duct thickening (n = 11). Serum IgG4 levels were elevated in 12 patients (75%), but not in any of the 25 patients with hilar CCC. Ten patients (63%) had a past or concurrent history of autoimmune pancreatitis (AIP). The significant infiltration of IgG4‐positive cells was observed with endobiliary or liver biopsy in 11 of 16 patients (69%) with ISC, but not in any patients with PSC or hilar CCC. Extrabiliary organ involvement, including sialadenitis, inflammatory pseudotumor of the liver and kidney, and retroperitoneal fibrosis, was present in seven patients. Marked improvement of biliary strictures and/or extrabiliary involvement was observed in all ISC patients after steroid therapy. Conclusions: ISC should be considered in the differential diagnosis of hilar/intrahepatic biliary strictures. Past or concurrent AIP or extrabiliary organ involvement strongly suggests the possibility of ISC. Significant infiltration of IgG4‐positive cells on endobiliary or liver biopsy specimens, and/or elevated serum IgG4 levels, highly support the diagnosis of ISC and provide the rationale for steroid therapy.     

Endoscopic ultrasound‐guided biliary drainage of hilar biliary obstruction

Only 20–30% of patients with hilar cholangiocarcinoma (CC) are candidates for potentially curative resection. However, even after curative (R0) resection, these patients have a disease recurrence rate of up to 76%. The prognosis of hilar cholangiocarcinoma (CC) is limited by tumor spread along the biliary tree leading to obstructive jaundice, cholangitis, and liver failure. Therefore, palliative biliary drainage may be a major goal for patients with hilar CC. Endoscopic retrograde cholangiopancreatography (ERCP) with stent placement is an established method for palliation of patients with malignant biliary obstruction. However, there are patients for whom endoscopic stent placement is not possible because of failed biliary cannulation or tumor infiltration that limits transpapillary access. In this situation, percutaneous transhepatic biliary drainage (PTBD) is an alternative method. However, PTBD has a relatively high rate of complications and is frequently associated with patient discomfort related to external drainage. Endoscopic ultrasound‐guided biliary drainage has therefore been introduced as an alternative to PTBD in cases of biliary obstruction when ERCP is unsuccessful. In this review, the indications, technical tips, outcomes, and the future role of EUS‐guided intrahepatic biliary drainage, such as hepaticogastrostomy or hepaticoduodenostomy, for hilar biliary obstruction will be summarized.     

使用增剪侧孔的PTCD引流管治疗高位胆道梗阻

目的探讨使用增剪侧孔的PTCD引流管治疗高位胆道梗阻的方法与疗效。方法 36例高位恶性梗阻性黄疸患者,其中肝门部胆管癌32例,肝转移癌4例。经皮穿刺一侧肝内胆管,增剪侧孔的PTCD外引流管通过闭塞段,猪尾型头端置于接近闭塞部的肝总管或胆总管,侧孔位于梗阻近段,实现全肝（Bismuth-CorletteⅠ、Ⅱ型梗阻）或一侧肝脏（Ⅲ、Ⅳ型梗阻）无肠液返流的内外引流。Ⅳ型梗阻病变累及肝段胆管时,引流管侧孔置于穿刺道所在肝管,头端通过闭塞段置于另一闭塞的肝段肝管,增强引流效果。结果 31例成功施行跨越闭塞段后置管,完成内外引流27例和多闭塞段的单引流管外引流4例,技术成功率86%。5例因导丝未能通过闭塞段,用常规方法行一侧肝叶或肝段胆管外引流。术后1周左右总胆红素平均值由术前（189±53）μmol/L降至（135±34）μmol/L,平均下降幅度（33±13）%;临床症状改善。结论应用增剪侧孔的引流管治疗高位胆道梗阻灵活、方便,近期疗效肯定,可选择做为临床常用方法。     

Surgery for hilar cholangiocarcinoma: the Johns Hopkins approach

Carcinoma of the hepatic duct confluence is the most common site of bile duct malignancies. Significant progress has been made in recent years in the diagnosis and treatment of this disease. The diagnosis is generally made in the jaundiced patient with contrast‐enhanced spiral computed tomography (CT) scanning followed by cholangiography. Percutaneous transhepatic cholangiography is favored over endoscopic retrograde cholangiography in that it better defines the proximal extent of the tumor involvement and allows placement of percutaneous transhepatic catheters. The preoperative placement of stents facilitates the surgical management, as well as decompressing the obstructed biliary tree. The operative technique at The Johns Hopkins Hospital involves resection of the hepatic confluence and reconstruction over Silastic stents. In patients in whom the hilar cholangiocarcinoma extends along either the right or left hepatic duct into the hepatic parenchyma, appropriate hepatic lobectomy is performed. A recent review of the Johns Hopkins experience has demonstrated that 67% of cholangiocarcinomas are located in the perihilar location. Fifty‐six percent of these patients were resectable for potential cure. Of the 109 resected patients, 4 patients (3.6%) died secondary to complications of sepsis. In the 109 patients with resected perihilar tumors, the 1‐, 3‐, and 5‐year survival was 68%, 30% and 11%, respectively. The median survival was 19 months. The addition of hepatic lobectomy did not alter the survival rate. Negative margins and negative lymph node status were associated with improved survival. Postoperative adjuvant radiation therapy did not provide benefit to patients with resected perihilar carcinoma.