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.     

Breathhold unenhanced and gadolinium-enhanced magnetic resonance tomography and magnetic resonance cholangiography in hilar cholangiocarcinoma

We assessed the imaging characteristics of hilar cholangiocarcinoma in magnetic resonance imaging (MRI) and magnetic resonance cholangiography (MRC). Breathhold MRI (T2-weighted turbo spin echo sequences, unenhanced T1-weighted gradient echo sequences, and gadolinium-enhanced fat-suppressed gradient echo sequences) and breathhold MRC (fat-suppressed two-dimensional projection images) performed in 12 patients with histologically confirmed hilar cholangiocarcinoma were retrospectively reviewed for morphological tumor characteristics and contrast enhancement patterns. MRC demonstrated a significant bile duct stenosis with intrahepatic bile duct dilatation in all cases except in one patient who received an endoprothesis prior to imaging. Hilar cholangiocarcinoma was diagnosed by MRC only in one patient and MRI and MRC in 11. Mass lesions were seen in nine patients and circumferential tumor growth in three, including the patient diagnosed by MRC only. The tumor appeared hypointense relative to liver parenchyma in 10 of 11 patients in unenhanced T1-weighted images. T2-weighted sequences showed isointense or only slightly hyperintense signal in 5 of 11 patients, 3 of whom demonstrated desmoplastic reactions by histology. The other 6 patients revealed strongly hyperintense signal intensities. Contrast enhancement was increased compared to liver in 5 of 11 patients and decreased in 6 of 11 patients. MRI with MRC seem to be a sensitive tools in the detection of hilar cholangiocarcinomas. The variable imaging characteristics are most probably related to the inhomogeneous histological appearance of this tumor entity.     

Diagnosis of cholangiocarcinoma

Cholangiocarcinoma is suspected based on signs of biliary obstruction, abnormal liver function tests, elevated tumor markers (carbohydrate antigen 19-9 and carcinoembryonic antigen), and ultrasonography showing a bile stricture or a mass, especially in intrahepatic cholangiocarcinoma. Magnetic resonance imaging (MRI) or computed tomography (CT) is performed for the diagnosis and staging of cholangiocarcinomas. However, differentiation of an intraductal cholangiocarcinoma from a hypovascular metastasis is limited at imaging. Therefore, reasonable exclusion of an extrahepatic primary tumor should be performed. Differentiating between benign and malignant bile duct stricture is also difficult, except when metastases are observed. The sensitivity of fluorodeoxyglucose positron emission tomography is limited in small, infiltrative, and mucinous cholangiocarcinomas. When the diagnosis of a biliary stenosis remains indeterminate at MRI or CT, endoscopic imaging (endoscopic or intraductal ultrasound, cholangioscopy, or optical coherence tomography) and tissue sampling should be carried out. Tissue sampling has a high specificity for diagnosing malignant biliary strictures, but sensitivity is low. The diagnosis of cholangiocarcinoma is particularly challenging in patients with primary sclerosing cholangitis. These patients should be followed with yearly tumor markers, CT, or MRI. In the case of dominant stricture, histological or cytological confirmation of cholangiocarcinoma should be obtained. More studies are needed to compare the accuracy of the various imaging methods, especially the new intraductal methods, and the imaging features of malignancy should be standardized.     

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.     

Endoscopic staging of hilar cholangiocarcinoma]

The prognosis of hilar cholangiocarcinoma is very poor due to its location and complicated anatomical characteristics. The bile duct cancer arising in the hilum easily invades the vascular structures and spreads along the bile duct. Complete curation could only be expected when curative resection of the hilar cholangiocarcinoma had been achieved. For the operability to be decided, the evaluation of longitudinal and vertical tumor extensions are important. Preoperative endoscopic staging work-up could be performed using endoscopic retrograde cholangiography (ERC), choledochoscopy, endoscopic ultrasonography (EUS) and intraductal ultrasonography (IDUS). ERC and choledochoscopic examinations have an advantage that these could take biopsy specimens. However ERC is not superior to either magnetic resonance cholangiography or percutaneous transhepatic cholangiography to make a better evaluation of the extent of the disease. Major problem of ERC is procedure-induced cholangitis, especially in Bismuth-Corlette type III and IV hilar cholangiocarcinoma. Percutaneous transhepatic choledochoscopic examination has an advantage that the stricture site could be examined directly with the availability of biopsy specimens. The diagnostic accuracy rates are different according to the morphological types of cholangiocarcinoma. EUS or IDUS could provide an information about the nodal involvement, the relationship with portal vein and the vertical extension of bile duct cancer. However, further study about the usefulness of EUS or IDUS would be needed in hilar cholangiocarcinoma. Above mentioned endoscopic examinations could be of help to decide the proximal margin of hilar cholangiocarcinoma. Each examination has its own limitations and advantages. Therefore appropriate combination of diagnostic modalities could be helpful to decide the best treatment option.     

Intraductal ultrasonography combined with percutaneous transhepatic cholangioscopy for the preoperative evaluation of longitudinal tumor extent in hilar cholangiocarcinoma

Background and Aim: In hilar cholangiocarcinoma, an accurate assessment of preoperative resectability is important to optimize surgical resection. We investigated the accuracy of the combination of intraductal ultrasonography (IDUS) and percutaneous transhepatic cholangioscopy (PTCS) for evaluating longitudinal extent in hilar cholangiocarcinoma. Methods: Patients diagnosed with hilar cholangiocarcinoma underwent multidetector computed tomography (MDCT) and magnetic resonance cholangiography (MRC) for tumor staging and Bismuth type. Percutaneous transhepatic biliary drainage was performed at the left or right bile duct of the liver section that was anticipated to be preserved in the surgical treatment. After tract dilation, PTCS with cholangioscope‐directed biopsy and IDUS were sequentially performed to evaluate Bismuth type. Surgical treatment was executed according to tumor staging and longitudinal tumor extent. Postoperative histological Bismuth types were compared to preoperative Bismuth types based on MDCT, MRC, PTCS with biopsy, and IDUS. Results: From June 2006 to November 2008, 25 patients with hilar cholangiocarcinoma were enrolled, with 20 of these patients evaluable. The accuracy of MDCT, MRC, PTCS with biopsy, and IDUS for the evaluation of Bismuth type was 80%, 84.2%, 90%, and 85.0%, respectively, in 20 patients, and 82.4%, 82.4%, 94.1%, and 88.2%, respectively, in 18 patients with Bismuth type IIIa, IIIb, or IV cancer. The accuracy of the combination of IDUS and PTCS with biopsy was 95% in 20 patients, and 100% in 18 with Bismuth type IIIa, IIIb, or IV cancer. Conclusions: The combination of IDUS and PTCS with biopsy was highly accurate for assessing Bismuth type and may help in the identification of an optimal surgical plan for the treatment of hilar cholangiocarcinoma, especially in Bismuth type IIIa, IIIb, or IV.     

Surgical management of cholangiocarcinoma

Biliary tract cancer affects approximately 7500 Americans each year. Tumors arising from the gallbladder are the most common; those of bile duct origin, or cholangiocarcinoma, are less frequently encountered, constituting approximately 2% of all reported cancers. Although cholangiocarcinoma can arise anywhere within the biliary tree, tumors involving the biliary confluence (i.e., hilar cholangiocarcinoma) represent the majority, accounting for 40 to 60% of all cases. Twenty to 30% of cholangiocarcinomas originate in the lower bile duct, and approximately 10% arise within the intrahepatic biliary tree and will present as an intrahepatic mass. Complete resection remains the most effective and only potentially curative therapy for cholangiocarcinoma. For all patients with intrahepatic cholangiocarcinoma and nearly all patients with hilar tumors, complete resection requires a major partial hepatectomy. Distal cholangiocarcinomas, on the other hand, are treated like all periampullary malignancies and typically require pancreaticoduodenectomy. Most patients with cholangiocarcinoma present with advanced disease that is not amenable to surgical treatment, and even with a complete resection, recurrence rates are high. Adjuvant therapy (chemotherapy and radiation therapy) has not been shown clearly to reduce recurrence risk.     

Gadoxetic acid magnetic-enhanced resonance imaging in the diagnosis of cholangiocarcinoma

The use of liver magnetic resonance imaging is increasing thanks to its multiparametric sequences that allow a better tissue characterization, and the use of hepatobiliary contrast agents. This review aims to evaluate gadoxetic acid enhanced magnetic resonance imaging in the diagnosis and staging of cholangiocarcinoma and its different clinical and radiological classifications proposed in the literature. We also analyze the epidemiology, risk factors in correlation with clinical findings and laboratory data.     

Three-dimensional imaging identified the accessory bile duct in a patient with cholangiocarcinoma

The development of diagnostic imaging technology, such as multidetector computed tomography (MDCT) and magnetic resonance cholangiopancreatography (MRCP), has made it possible to obtain detailed images of the bile duct. Recent reports have indicated that a 3-dimensional (3D) reconstructed imaging system would be useful for understanding the liver anatomy before surgery. We have investigated a novel method that fuses MDCT and MRCP images. This novel system easily made it possible to detect the anatomical relationship between the vessels and bile duct in the portal hepatis. In this report, we describe a very rare case of extrahepatic cholangiocarcinoma associated with an accessory bile duct from the caudate lobe connecting with the intrapancreatic bile duct. We were unable to preoperatively detect this accessory bile duct using MDCT and MRCP. However, prior to the second operation, we were able to clearly visualise the injured accessory bile duct using our novel 3D imaging modality. In this report, we suggest that this imaging technique can be considered a novel and useful modality for understanding the anatomy of the portal hepatis, including the hilar bile duct.     

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.     

Mucin-producing intrahepatic cholangiocarcinoma with portal vein thrombosis

Mucin-producing intrahepatic cholangiocarcinoma is rare. Computed tomographic scan and magnetic resonance imaging showed a well-defined tumor with marked dilatation of the left intrahepatic bile duct and portal vein thrombosis. We performed extended left hepatectomy and portal vein thrombectomy. A massive amount of mucin was observed in the left intrahepatic bile duct. Histological examination revealed noninvasive and well differentiated tubular adenocarcinoma.     

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.     

Primary biliary non-Hodgkin's lymphoma: A case report

Rationale:Primary biliary non-Hodgkin''s lymphoma (PBNHL) is a rare disease with only 41 cases reported since 1982. The incidence of PBNHL in patients with malignant cholangiocarcinoma was 0.6%, and PBNHL accounted for 0.4% of extranodal non-Hodgkin''s lymphoma, and only 0.016% of all non-Hodgkin''s lymphoma cases.Patient concerns:We present a rare case of PBNHL in a 59-year-old female who had jaundice for 3 days with weight loss and Epstein-Barr virus infection. Initial computed tomography and magnetic resonance imaging showed diffuse thickening wall of bile ducts with corresponding lumen stenosis, blurred fat space around the portal vein, lymphadenopathy, and a normal spleen. These manifestations and images were similar to hilar cholangiocarcinoma. So, the diagnosis of hilar cholangiocarcinoma was initially considered.Diagnoses:Postoperative pathology confirmed the final diagnosis was PBNHL.Interventions:The patient and her family requested to clarify the histologic diagnosis by laparotomy biopsy. Because the biopsy result could not be defined during operation, then right hemihepatectomy and choledochojejunostomy were performed. She did not receive any antitumor treatment.Outcomes:One month after the patient''s first examination, both computed tomography and magnetic resonance images showed diminished stenosis of common bile duct and left hepatic duct, but a new mass in segment IV of liver was observed. Unfortunately, the patient died due to disease progression.Lessons:This case reminds us that although PBNHL is rare, making accurate diagnosis difficult preoperatively, PBNHL should be considered when encountering a case with Epstein-Barr virus infection and those typical imaging findings.     

Hilar cholangiocarcinoma]

Hilar cholangiocarcinoma is defined as a malignant neoplasm involving right and left main hepatic ducts and/or its confluency. The disease is more prevalent in East Asia including Korea than Western countries and it may be due to the facts that hepatolithiasis, clonorchiasis and congenital anomaly of bile ducts are more frequent in this region. In this review, we will discuss about radiologic, endoscopic, and TNM staging along with preoperative assessment for surgical strategy in patients with hilar cholangiocarcinoma.     

Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis?

BACKGROUND: Autoimmune pancreatitis has been designated as sclerosing pancreatocholangitis, because this disease shows a high prevalence of bile-duct lesions. We present herein the clinical characteristics of unusual cases that show dominant bile-duct lesions and mimicking infiltrating hilar cholangiocarcinomas. METHODS: Clinical and pathologic findings of 3 patients with immunoglobulin (Ig) G4 related sclerosing cholangitis who had no apparent pancreatic lesions comparable with autoimmune pancreatitis were analyzed. OBSERVATIONS: All patients were middle-aged or elderly individuals with slightly elevated serum IgG4 concentrations and showed long-segment narrowing of the bile-duct system, mimicking infiltrating hilar cholangiocarcinoma without significant pancreatic change. The first patient was treated with a corticosteroid, resulting in amelioration of the narrowing of the bile duct. The second patient underwent surgery based on a diagnosis of cholangiocarcinoma. In the third patient, the bile-duct stricture reversed spontaneously 1 month after the drainage procedure. Pathologic findings of the bile ducts for all patients disclosed significant lymphoplasmacytic infiltration, including abundant IgG4-bearing plasma cells. CONCLUSIONS: The use of IgG4 immunostaining in biopsy specimens of the bile duct may identify the presence of corticosteroid-responsive lymphoplasmacytic sclerosing cholangitis.     

A case of follicular cholangitis mimicking hilar cholangiocarcinoma

Follicular cholangitis is a sclerosing cholangitis with hilar biliary stricture that must be differentiated from both immunoglobulin G4-related sclerosing cholangitis and primary sclerosing cholangitis. This disorder is extremely rare and difficult to distinguish from hilar biliary cholangiocarcinoma. We report here a case of a Japanese female patient in her 60s with this disease. The patient visited a family doctor for itching and general fatigue. Blood examination showed elevated hepatobiliary enzyme levels. Various imaging studies showed dilation of the bilateral intrahepatic bile duct and wide stenosis from the proximal bile duct to the right and left hepatic duct. They also showed the enlargement of multiple lymph nodes in the hepatoduodenal ligament, periaorta, and mesocolon. Based on endoscopic retrograde cholangiopancreatography-directed brush cytology, we diagnosed this patient with hilar cholangiocarcinoma and performed left trisegmentectomy of the liver. The pathology results showed that the wall from the bilateral hepatic duct to the proximal bile duct had thickened irregularly with dense fibrosis and a marked formation of lymph follicles. The mucosal epithelia did not have malignant findings. The diagnosis was follicular cholangitis. This case indicates that follicular cholangitis should be considered as a differential diagnosis of hilar biliary stricture.     

Surgical management of resectable hilar cholangiocarcinoma

Hilar cholangiocarcinoma is a cancer with a poor long-term survival rate (20% at five years), even after curative resection (R0: surgical margins free of tumour). Recent advances have been made in the preoperative management of these patients: clinical imaging, such as magnetic resonance cholangiography, biliary drainage according to the type of hilar obstruction or laparoscopic assessment before portal vein embolization. Extended hemihepatectomy has recently been recognized as the standard curative treatment for hilar bile-duct cancer with concomitant resection of the portal-vein bifurcation as a radical resective procedure. Neoadjuvant-photodynamic therapy could be a new approach to treating hilar cholangiocarcinoma decreasing tumour margins, which is a major prognostic factor.     

Prognostic impact of the Bismuth-Corlette classification:Higher rates of local unresectability in stageⅢb hilar cholangiocarcinoma

Background:The Bismuth-Corlette(BC)classification is used to categorize hilar cholangiocarcinoma by proximal extension along the biliary tree.As the right hepatic artery crosses just behind the left bile duct,we hypothesized that BCⅢb tumors would have a higher likelihood of local unresectability due to involvement of the contralateral artery.Methods:A retrospective review of a prospectively maintained database identified patients with hilar cholangiocarcinoma taken to the operating room for intended curative resection between April 2008 and September 2016.Cases were assigned BC stages based on preoperative imaging.Results:Sixty-eight patients were included in the study.All underwent staging laparoscopy after which 16 cases were aborted for metastatic disease.Of the remaining 52 cases,14 cases were explored and aborted for locally advanced disease.Thirty-eight underwent attempt at curative resection.After exclud-ing cases aborted for metastatic disease,the chance of proceeding with resection was 55.6%for BCⅢb staged lesions compared to 80.0%of BCⅢa lesions and to 82.4%for BCⅠ-Ⅲa staged lesions(P<0.05).About 44.4%of BCⅢb lesions were aborted for locally advanced disease versus 17.6%of remaining BC stages.Conclusions:When hilar cholangiocarcinoma is preoperatively staged as BCⅢb,surgeons should antici-pate higher rates of locally unresectable disease,likely involving the right hepatic artery.     

Potential utility of magnetic resonance cholangiography in the management of endoscopic biliary drainage tubes

An 80-yr-old woman with advanced hilar cholangiocarcinoma underwent a placement of endoscopic biliary drainage (EBD) tube from the common hepatic to common bile duct through the stricture. Magnetic resonance cholangiography clearly demonstrated the later dislocation and obstruction of the EBD tube. The present case suggests that magnetic resonance cholangiography may be a potentially useful tool in the management of EBD tubes.     

Obstructive jaundice and melena caused by hemocholecyst: A case report

A hemocholecyst (HC) is a clot-filled gallbladder caused by bleeding into its lumen. Obstructive jaundice caused by the compression of HC to the hilar biliary tract is likely to be misdiagnosed as cholangiocarcinoma and is extremely rare. We herein report a case of obstructive jaundice and melena caused by HC. A 57-year-old male patient presented with right upper quadrant pain associated with icteric sclera and melena was suspiciously diagnosed as having malignant cholangiocarcinmoa by abdominal ultrasonography, computed tomography and magnetic resonance imaging. Laparotomy found a hematoma in the gallbladder. The hematoma spread to the left hepatic lobe forming an exogenous mass which compressed the hilar biliary tract. Radical cholecystectomy and bile duct exploration with T-tube drainage were performed. Histopathological examination revealed massive necrosis of the gallbladder mucosa with inflammatory cells infiltration as well as intraluminal hematoma formation. One month after operation, a T-tube cholangiography revealed a normal biliary tree. We suggest that HC should be considered in patients with obstructive jaundice and melena after common causes are ruled out.