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.     

Cholangiocarcinoma:principles and current trends

BACKGROUND:Cholangiocarcinoma(CCA)is a lethal cancer of the biliary epithelium,originating from the liver(intrahepatic),at the confluence of the right and left hepatic ducts(hilar)or in the extrahepatic bile ducts.It is a rare malignancy associated with poor prognosis.DATA SOURCES:We searched the PubMed/MEDLINE database for relevant articles published from 1989 to 2008.The search terms used were related to cholangiocarcinoma and its treatment.Although no language restrictions were imposed initially,for the...     

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.     

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.     

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.     

Altered Mucin Gene Expression in Stone-Containing Intrahepatic Bile Ducts and Cholangiocarcinomas

Neoplastic transformation of epithelial cells is commonly associated with alterations in the synthesis and structures of mucin. Mucin protein epitopes and mRNA levels were frequently altered in adenocarcinomas compared to corresponding normal tissues. Clinically, hepatolithiasis has been regarded as a risk factor for cholangiocarcinoma. The aims of this study were to determine the possible alteration of mucin gene expression in stone-containing intrahepatic bile ducts and cholangiocarcinomas and to try to predict whether or not hepatolithiasis has a predisposition to development of cholangiocarcinoma. In situ hybridization with DIG-tailed oligonucleotides was performed on sections of paraffin-embedded tissues of stone-containing intrahepatic bile ducts, cholangiocarcinomas, and normal controls to identify the expression of MUC2, MUC3, MUC4, MUC5B, and MUC5AC in nonneoplastic and neoplastic biliary epithelium. The findings showed that (1) while multiple diverse mucin genes were expressed in the biliary epithelium, MUC3 and MUC5B mRNA were the main mucin genes expressed in the biliary epithelium of stone-containing intrahepatic bile ducts and normal controls; (2) absent or decreased expression of MUC2, MUC3, and MUC5B of mRNA was found in cholangiocarcinomas in contrast to nonneoplastic biliary epithelium; and (3) increased expression of MUC4 and MU5AC of mRNA was found in cholangiocarcinomas and the biliary epithelium, especially for dysplastic cells of stone-containing intrahepatic bile ducts compared with normal controls. In this study, using in situ hybridization we demonstrated that neoplastic transformation of the biliary epithelium is accompained by alterations in mucin gene expression, the altered mucin gene expression in dysplastic cells of stone-containing intrahepatic bile ducts may reflect a higher potential for malignant transformation in these cells, and it could be a precursor of cholangiocarcinoma in the presence of hepatolithiasis.     

Inflammatory pseudotumor of the liver with primary sclerosing cholangitis

Inflammatory pseudotumor (IPT) of the liver is a rare benign variant of hepatic masses, and its exact etiology has not been elucidated. We report a case of IPT associated with primary sclerosing cholangitis (PSC). The patient was a 50-year-old man admitted to our hospital because of jaundice. Abdominal ultrasonography (US) and computed tomography showed multiple dilations of the intrahepatic bile ducts and multiple masses in the liver. On magnetic resonance imaging, the masses were slightly hypointense on T1-weighted images and slightly hyperintense on T2-weighted images. On T1-weighted images after the bolus infusion of Gd chelate, the masses had no contrast enhancement, and they were hypointense in the arterial phase and portal venous phase. However, they were slightly enhanced and became almost isointense relative to the surrounding normal liver parenchyma in the delayed phase. Endoscopic retrograde cholangiography demonstrated multiple irregular strictures and dilations of the intrahepatic bile ducts. Angiography demonstrated no abnormal findings, but, interestingly, subsequent dynamic CO₂-enhanced US showed a strongly hyperechoic string, indicating that an artery had penetrated through the hypoechoic mass. A US-guided percutaneous needle biopsy revealed that the lesions were morphologically comparable to IPT. After cholangiography and microscopic analysis of the tumor, the final diagnosis was determined to be IPT of the liver with PSC. A number of previous reports have suggested a possible relationship between IPT and PSC, based on pathological findings. This report confirmed, based on clinical findings, that PSC is one of the causes of hepatic IPT. Received: May 12, 1999 / Accepted: October 22, 1999     

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.     

Multicystic biliary hamartoma mimicking intrahepatic cholangiocarcinoma: report of a case

Multicystic biliary hamartoma (MCBH) is a rare cystic disease of the liver. A 69-year-old man was referred to our hospital with radiographic abnormality. Physical examination of the patient was unremarkable, and he denied any previous medical, travel, or surgical history. An abdominal computed tomography (CT) scan demonstrated a 3-cm low-density lesion in segment 3 of the liver, with dilation of the intrahepatic bile duct. The peripheral site of this lesion was slightly enhanced in the arterial phase. In the portal phase, the peripheral site was enhanced more clearly and showed a honeycomb-like dilated bile duct. Ultrasonography also revealed that the lesion was an irregularly shaped mass. On magnetic resonance imaging (MRI), T1-weighted images revealed a low-density mass and T2-weighted images revealed a dappled-density mass with honeycomb-like dilated bile duct and dilation of major intrahepatic bile duct. The patient was diagnosed with intrahepatic cholangiocarcinoma (ICC) and underwent left hepatectomy. However, pathological findings revealed that the lesion was MCBH. Our case highlights the potential difficulties in differentiating between MCBH and ICC under such circumstances.     

A case of percutaneous and transpapillary placements of expandable metallic stents in a patient with cholangiocarcinoma at the hilum of the liver

A 76-year-old woman was admitted with obstructive jaundice. US and MR cholangiopancreatography (MRCP) revealed an inoperative cholangiocarcinoma, 3 cm in diameter at the hilum of the liver, the obstruction of the hepatic duct bifurcation and the separation of bilateral hepatic bile ducts. Percutaneous transhepatic biliary drainage (PTBD) was performed from bilateral hepatic bile ducts. The right PTBD tube was spontaneously extubated. We could not succeed in performing internal biliary drainage across the hilar malignant stricture from a left hepatic bile duct, because of bad angulation. Transpapillary insertion into the common bile duct (CBD) was extremely difficult due to the collapse of the CBD. Endoscopic sphincterotomy (EST) after precutting method was performed. Although we performed the ballooned dilatation of malignant stricture and the insertion of a self-expandable metallic stent (EMS) into a right hepatic bile duct transpapillary. After dilatation of the hilar malignant stricture by the initial EMS, we inserted a guidewire into the CBD through the wire mesh of a stent from the left PTBD tube. We could insert the second EMS from a left hepatic bile duct to the CBD transhepatically, using a dilator and a dilating balloon. Finally, we performed the ballooned dilatation from bilateral hepatic bile ducts to the CBD transpapillary. She was discharged after bilateral internal biliary drainages, successfully.     

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.     

Optical coherence tomography of the biliary tree during ERCP.

BACKGROUND: Optical coherence tomography (OCT) is a high-resolution imaging technique that produces cross-sectional images in vivo. This is a report of the first use of OCT to acquire images of the biliary tree during ERCP. METHODS: A 2.6-mm diameter OCT catheter delivered through the accessory channel of a duodenoscope was used to acquire images of the intrahepatic and extrahepatic bile ducts in 5 patients. Histology-correlated OCT images of 5 cadaveric livers, each imaged at 3 sites, were used to guide interpretation of the in vivo images. RESULTS: Biliary ductal epithelium and subepithelial structures, including peribiliary glands, vasculature, and hepatic parenchyma, were visualized in vivo. The papillary architecture of cholangiocarcinoma was identified by OCT. There were no OCT-related complications. CONCLUSIONS: High-resolution OCT imaging of the biliary tree is feasible and may provide useful diagnostic information during ERCP.     

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.     

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.     

Parenchymal changes of the liver in cholangiocarcinoma: CT evaluation.

We evaluated parenchymal changes of the liver in 92 patients (41 peripheral types and 51 hilar types) with cholangiocarcinomas studied by bolus-enhanced computed tomography (CT). In 39% of patients with the peripheral type, a wedge-shaped increased enhancement of the liver was observed peripheral to the tumor on bolus-enhanced CT. Tumor was observed in all cases. In 58.8% of patients with the hilar type, a segmental or lobar increased degree of enhancement of the liver was observed, but the tumor was demonstrated in only 58.8%. Atrophy was accompanied by areas of increased enhancement in 80% of hilar type and 25% of peripheral type. Areas of increased degree of enhancement corresponded to a wedged-shaped perfusion defect on CT during arterial portography. On magnetic resonance imaging (MRI), those lesions showed hyperintensity on T2-weighted images. Most of these changes were considered to be due to reversible hepatic parenchymal ischemia secondary to portal vein invasion by the tumor.     

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。     

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.     

Clinicopathological studies of mucin-producing cholangiocarcinoma

Cholangiocarcinomas that produced clinically recognizable mucin (mucin-producing cholangiocarcinomas) were studied in ten patients. These ten represented 5.8% of all cholangiocarcinomas resected at our department. All tumors arose from the intrahepatic bile ducts. Macroscopically, the ten tumors were classified as: “ductectatic type” (n=3), in which papillary tumors developed in diffusely dilated intrahepatic bile ducts; “cystic type” (n=5), in which a large cystic tumor was present in the liver; and “intermediate type” (n=2), characterized by a large cystic lesion and a solid mass that infiltrated the liver parenchyma. Histologically, four tumors remained localized to the mucosal layer and six invaded the fibrous layer and the liver parenchyma. The invasion pattern of the six invasive cancers resembled that of the most common type of cholangiocarcinoma. Superficial spread of the cancer contiguous to the primary tumor was observed in four of the ten patients.     

MRCP and MRI findings in 9 patients with autoimmune pancreatitis

AIM: To evaluate magnetic resonance cholangiopancre-atography (MRCP) findings in conjunction with magnetic resonance (MR) images in autoimmune pancreatitis (AIP) patients. METHODS: Nine patients with AIP underwent MRI, MRCP, endoscopic retrograde cholangiopancreatography (ERCP), computed tomography, and ultrasonography. The MRCP and MR images taken before and after steroid therapy were reviewed and compared with other imaging modalities. The MRCP findings of the AIP cases were compared to those of 10 cases with carcinoma of the head of the pancreas. RESULTS: On MRCP, the narrowed portion of the main pancreatic duct noted on ERCP was not visualized, while the non-involved segments of the main pancreatic duct were visualized. The degree of upstream dilatation of the proximal main pancreatic duct was milder than that seen in cases of pancreatic carcinoma. Stenosis or obstruction of the lower bile duct was detected in 8 patients. MR images showed enlargement of the pancreas with decreased signal intensity on T1-weighted MR images, increased signal intensity on T2-weighted MR images, and, in 3 patients, a hypointense capsule-like rim. After steroid therapy, the previously not visualized portion of the main pancreatic duct was seen, along with improvement of the bile duct stenosis. Pancreatic enlargement decreased, and the abnormal signal intensity on both T1- and T2-weighted MR images became isointense. CONCLUSION: MRCP cannot differentiate irregular narrowing of the main pancreatic duct seen with AIP from stenosis of the main pancreatic duct seen with pancreatic carcinoma. However, MRCP findings in conjunction with MR imaging of pancreatic enlargement that shows abnormal signal intensity on T1- and T2-weighted MR images are useful in supporting a diagnosis of AIP.     

Right hepatectomy with resection of caudate lobe and extrahepatic bile duct for hilar cholangiocarcinoma

En-bloc liver resection with caudate lobectomy (segmentectomy 1) is the standard procedure for hilar cholangiocarcinoma. Although its surgical mortality has been reduced below 5%, it is still a potentially hazardous operation. Complete tumor resection with negative surgical margins and safe reconstruction of bilio-enteric continuity are two principles of the surgical treatment of hilar cholangiocarcinoma. Surgeons must pay attention to the variation of the hilar structures including portal veins, hepatic arteries, and bile ducts. Three-dimensional imaging is beneficial not only for understanding anatomical variations but also for preoperative simulations. Since the U-point can be identified by both preoperative imaging and intraoperative inspection, it can be used as the landmark for the hepatectomy and the dissection point of the hilar plate. The hanging maneuver might be useful for both hepatic parenchymal dissection and bile duct dissection just right of the U-point. For safe biliary reconstruction, stay sutures in the anterior wall and transanastomotic stents may be helpful.