08.01.2014

Medicine for bile duct cancer prognosis

Cancer of the ampulla of vater often metastasizes into the lymph nodes, and the five-year survival rate is only about 40 percent. When food is digested, it passes from the stomach to the small intestine, then into the large intestine. The ampulla of vater carries bile from the common bile duct, as well as secretions from the pancreas through the pancreatic duct into the small intestine. Disney engineers designed special paints to help camouflage unsightly park features like utility boxes and back doors. Also known as the hepatopancreatic ampulla, the ampulla of vater carries bile from the common bile duct, as well as secretions from the pancreas through the pancreatic duct, into the small intestine.
One result of secretions not flowing smoothly through the digestive system can be pancreatitis. Rarely, cancer can develop in the hepatopancreatic ampulla, with symptoms including jaundice and pain. If you want to improve your digestive system the number one thing you can do is eat a lot of fruits and vegetables. Patients with partial bile duct obstruction have elevated serum alkaline phosphatase (ALP) and gamma-glutamyl transpeptidase (GGT, GGTP). The prothrombin time (PT) and international normalized ratio (INR) may be prolonged and can usually be normalized with parenteral administration of vitamin K. Other laboratory abnormalities sometimes observed are anemia, elevated amylase and lipase levels, and an elevated erythrocyte sedimentation rate (ESR) and lactic dehydrogenase (LDH) level. One study found that serum trypsinogen-2 is a useful marker for diagnosing patients with cholangiocarcinoma.[20] It is unclear whether trypsinogen-2 could be a new method to screen PSC or whether tumor marker combinations would be more useful.
In patients presenting acutely with features of cholangitis, the initial radiologic study should be an urgent right upper quadrant ultrasonogram.
Endoscopic retrograde cholangiopancreatographic image of a cholangiocarcinoma at the bifurcation of the right and left hepatic ducts (Klatskin tumor).
Endoscopic retrograde cholangiopancreatographic cholangiogram demonstrating a long bile duct stricture that represents external compression by gallbladder cancer. Endoscopic retrograde cholangiopancreatographic image of a cholangiogram in a patient with cholangiocarcinoma whose condition has been treated with a metal stent.
Endoscopic retrograde cholangiopancreatographic cholangiogram of a solitary benign stricture of the distal bile duct.
Endoscopic retrograde cholangiopancreatographic cholangiogram demonstrating an isolated mid-hepatic duct stricture as a result of pancreatic cancer.
Endoscopic retrograde cholangiopancreatographic cholangiogram demonstrating diffuse stricturing of the intrahepatic ducts that is consistent with primary sclerosing cholangitis. Ultrasonography (US) is generally considered the imaging modality of choice for the initial screening of biliary disorders.[21] US does not use ionizing radiation to create the image, and it is the least invasive radiologic technique for imaging the biliary tract. US can accurately detect dilatation of intrahepatic and extrahepatic bile ducts, thus providing indirect evidence for the presence of bile duct strictures.
Cholangiocarcinoma and PSC may cause biliary obstruction without ductal dilatation, and some benign strictures with partial obstruction may not be associated with biliary dilatation. Spiral computed tomography (SCT) and multidetector CT (MDCT) scanning are highly sensitive for the diagnosis of biliary obstruction, particularly when these modalities are performed with oral and intravenous contrast agents. Similar to US, CT scanning also helps detect intrahepatic and extrahepatic bile duct dilatation; however, the main value of CT scanning is its ability to detect the site of obstruction with greater accuracy than US and to help predict the cause of obstruction, especially malignant obstruction. CT cholangiography scanning is another technique that rivals endoscopic retrograde cholangiopancreatography (ERCP) in delineating biliary tract abnormalities but has not achieved widespread use because of some adverse reactions to the contrast material. CT scanning is superior to US in visualizing the distal CBD area, because gas artifacts may obscure this region when examined by US.
Other important areas that can be seen better on CT scans are the porta hepatis, pancreas, and liver parenchyma. Since its introduction, magnetic resonance cholangiopancreatography (MRCP) has rapidly become an important tool for visualizing the biliary system. MRCP is as sensitive as ultrasonography (US) for helping detect cholelithiasis and is superior to US for helping diagnose common bile duct (CBD) stones, malignant biliary obstruction, and benign pancreatic disease (see image below). Multiple small bile duct stones seen on magnetic resonance cholangiopancreatography (MRCP).
The presence of biliary dilatation can be accurately detected by MRCP in 97-100% of patients. In one study, sensitivity for the detection of any abnormality was 89% and the specificity was 92%; for the detection of malignancy, sensitivity was 81% and specificity was 100%. MRCP is also very useful in helping identify cholangiocarcinoma, which characteristically appears as enhancement in delayed images. Benign strictures due to sclerosing cholangitis are multifocal and alternate with slight dilatation or normal-caliber bile ducts, producing a beaded appearance.
Dilatation of both the pancreatic and bile ducts using MRCP is highly suggestive of a pancreatic head malignancy.
A hepatic iminodiacetic acid (HIDA) scan is a radionuclide scanning technique commonly used for the diagnosis of acute cholecystitis and biliary leaks. However, HIDA scans are insensitive for helping detect biliary dilatation or the site and cause of bile duct obstruction. Cytologic sampling is best performed by brushing the bile duct stricture during ERCP or percutaneous transhepatic cholangiography (PTC).
Routine cytology and histology yield a high specificity but low sensitivity for determining the etiology of bile duct strictures. DIA and FISH enhance the accuracy of standard techniques in the evaluation of indeterminate bile duct strictures, allowing the diagnosis of malignancy in a substantial number of patients with false-negative cytology and histology. Cytologic brushing of bile duct strictures is usually performed with wire guidance across the stricture.
Endoscopic retrograde cholangiopancreatography (ERCP) has been used for the examination of the pancreaticobiliary region for more than 30 years, and remains the criterion standard.
The endoscope used for ERCP is a side-viewing duodenoscope that has the capacity to control the direction of catheters as they exit the instrument channel of the scope. ERCP is a valuable technique in biliary disease because other diagnostic maneuvers (eg, sphincter of Oddi manometry) and therapeutic interventions (eg, stone extraction, biliary drainage, stent placement) can be carried out at the same time as the primary diagnosis.
ERCP can help detect intrahepatic and extrahepatic biliary dilatation, stones, and the site of bile duct stricture with the highest sensitivity and specificity (both approximately 90-100%). ERCP is also the criterion standard for the diagnosis of AIDS-related cholangitis and is essential for differentiating PSC from AIDS-related cholangitis.
Nevertheless, ERCP is associated with significant complications, including pancreatitis, bleeding, perforation, infection, and cardiopulmonary depression from conscious sedation.
Endoscopic ultrasonography (EUS) involves the use of echoendoscopes, which have an US transducer mounted at the end of a side-viewing or oblique-viewing endoscope. The linear-array EUS system also has color Doppler capability, enabling the endosonographer to be able to differentiate between vascular and fluid-filled structures. The instrument also has a small biopsy channel for fine-needle aspiration (FNA) and fine-needle injection. The pancreas, common bile duct (CBD), and the gallbladder are in close proximity to the distal stomach and the duodenum and can be viewed. EUS and EUS-guided FNA is a sensitive technique for the diagnosis and staging of cholangiocarcinoma and gallbladder, ampullary, and pancreatic cancer because it can also help detect the involvement of regional lymph nodes and vascular invasion by the tumor (an advantage over ERCP in this regard). EUS is also superior to computed tomography (CT) scanning for tumor, node, and metastases (TNM) staging of luminal and pancreaticobiliary malignancies. In more recent years, EUS-guided biliary stenting has become possible with the availability of EUS-guided stents.
Since the 1960s, percutaneous transhepatic cholangiography (PTC) has been used for the diagnosis and treatment of biliary tract disorders. The technique consists of introducing a 22- or 23-gauge needle through the skin in the right ninth or tenth intercostal space in the midaxillary line and advancing into the liver parenchyma under fluoroscopic guidance.
Indications for PTC in bile duct strictures are the presence of biliary-enteric anastomosis (eg, Roux-en-Y anastomosis with hepaticojejunostomy, choledochojejunostomy, Billroth II gastrectomy), the presence of complex hilar strictures, or when ERCP is unsuccessful. Therapeutic intervention, including biliary drainage, dilation of benign bile duct strictures, extraction of biliary tract stones, or placement of a stent across a malignant stricture also can be performed at the same time (see images below). This image is an example of an intraoperative cholangiogram performed during a laparoscopic cholecystectomy. Percutaneous transhepatic cholangiogram with balloon dilation of a postoperative bile duct stricture. Complications, including sepsis, bile leak, intraperitoneal hemorrhage, hemobilia, hepatic and perihepatic abscess, pneumothorax, and skin infection and granuloma at the catheter entry site, can occur in as many as 10% of cases. In postsurgery patients with an external biliary fistula or T tube, contrast medium can be injected into the biliary system through the tube or the fistula.
This study can precipitate cholangitis; therefore, patients should receive antibiotic prophylaxis. Surgically resected segments of the biliary tree will show the etiology of the bile duct stricture.
In cholangiocarcinoma, there will be evidence of adenocarcinoma in the cross-sectional histology of the bile duct. Periductal lymphocytic and plasma cell infiltrate that is consistent with autoimmune cholangiopathy.
Staging systems have been developed for bile duct strictures due to operative trauma, cholangiocarcinoma, and HIV disease. The modified Bismuth and Corlett classification of hilar cholangiocarcinoma is the most widely adopted anatomic classification of this tumor. Type 1: The confluence of the hepatic ducts is not involved, but the tumor is generally within 2 cm of the hilum.
Type 3a: The confluence is involved, with extension of the tumor into the right hepatic duct.
Type 3b: The confluence is involved, with extension of the tumor into the left hepatic duct.
Sclerosing cholangitis: This pattern is observed in 20% of patients and is characterized by focal strictures and dilatations involving intrahepatic and extrahepatic bile ducts. Combined papillary stenosis and sclerosing cholangitis: This is the most common pattern of HIV cholangiopathy and is observed in more than 50% of cases. Long strictures of extrahepatic bile ducts: This pattern is observed in approximately 15% of patients.
Benign distal common bile duct stricture seen during a cholecystostomy injection in an elderly male. Medscape's clinical reference is the most authoritative and accessible point-of-care medical reference for physicians and healthcare professionals, available online and via all major mobile devices.
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Cancer of the biliary systema€”including the gallbladder, bile ducts within the liver and bile ducts extending outside of the liver that drain into the intestinea€”is relatively uncommon, affecting about 9,000 individuals in the United States annually.
Distinguishing cholangiocarcinoma from mimicking conditions is imperative in guiding the appropriate treatment.
SurgicalFor surgical interventions, cholangiocarcinomas are divided into intrahepatic and extrahepatic bile duct cancers. Hepatobiliary and pancreas diseases - disorders of the liver, bile ducts, gall-bladder and pancreas - form a complex set of medical problems whose treatment often requires equally challenging surgical procedures. California Pacific Medical Center, part of the Sutter HealthOpens new window network, offers specialized care in liver disease and liver transplant. Bile duct cancer represents a rare malignancy that can occur anywhere along the intrahepatic or extrahepatic biliary tree. Although cholangitis is a rare initial presentation, the rate of both its occurrence and that of bactobilia increases significantly with either endoscopic or percutaneous biliary tract instrumentation. Certain pathological conditions related to acute or chronic biliary epithelial injury appear to predispose to the development of cholangiocarcinomas 40% of autopsy specimens and 10% to 30% of liver explants after their transplantation from patients with primary sclerosing cholangitis (PSC) contained occult foci of cholangiocarcinoma (14).
Nevertheless, according to most authorities, cholangiography is mandatory when noninvasive studies suggest malignant biliary obstruction (3). Selection of patients who are "fit” to undergo radical surgery is sometimes different. Other modifications of the Bismuth-Corlette classification system have been suggested but have not been widely accepted. Additional prognostic factors that have been reported include histological variant of the tumor, presence of a blood vessel, lymphatic and perineural invasion, presence of lymph node involvement, and involvement of tumor margins. Before formal laparotomy, staging laparoscopy can be performed to identify lymphatic or occult peritoneal metastases not imaged by preoperative studies (3). Preoperative placement of percutaneous transhepatic catheters (PTCs) is part of standard management according to most authorities (3). Pancreaticoduodenectomy (Whipple´s procedure) is carried out for distal third bile duct tumors and ampullary cholangiocarcinomas. Middle third tumors are removed by resecting the extrahepatic biliary tree from above the duodenum to the base of the liver along with a lymphadenectomy of the porta hepatis. The surgical management of hilar tumors (HCCA, Klatskin tumors) initially involves the dissection of extrahepatic biliary structures (Figure 3). Apart from this "traditional” approach, most specialists have become more aggressive, including hepatic resection for those tumors. Occasionally, even more extensive procedures may be necessary, including back table ex vivo dissection.
Table 5 depicts selected surgical series of cholangiocarcinoma patients with respective clinical outcomes. Another controversial approach to the management of HCCA is orthotopic liver transplantation (OLT).


Patients with unresectable tumors and those who are not candidates for resection should have biliary drainage tubes placed either percutaneously or endoscopically. Unresectable lower third tumors are bypassed by end-to-side choledochojejunostomy or cholecystojejunostomy, which can be performed laparoscopically (3).
The patient receives an intravenous injection of a radioactive material called hydroxy iminodiacetic acid (HIDA). It will also pass into the common bile duct and enter the small intestine, from which it eventually makes its way out of the body in the stool. The HIDA scan should be done when ultrasound is not diagnostic and when there is a clinical diagnosis of acute cholecystitis. Usually, HIDA scans are ordered for patients who are suspected of having an obstruction in the biliary tract, most commonly those who are thought to have a stone blocking the cystic duct leading out of the gallbladder. Such a scenario is consistent with acute cholecystitis, which often requires surgical removal of the gallbladder. In cholecystitis, HIDA will appear in the bile ducts, but it will not enter the cystic duct or the gallbladder -- a finding that indicates obstruction. If the HIDA enters the bile ducts but does not enter the small intestine, then an obstruction of the bile duct (usually due to stones or cancer) is suspected.
HIDA scans can be falsely positive when the gallbladder is not filling in the absence of cholecystitis. Along the way, various acids and other substances interact with the food to break it down and make it possible for the body to absorb the nutrients it provides. Several sphincters ensure the secretions are directed to the right place and prevent the contents of the small intestine from flowing back out through the ampulla.
The serum of patients with clinically apparent jaundice shows increases in total and conjugated bilirubin.
Total cholesterol and lipoprotein levels may be elevated in patients with chronic cholestatic disorders. Again, in malignant strictures causing only partial obstruction (eg, Klatskin tumor), a rise in the alkaline phosphatase level may not be accompanied by a rise in the bilirubin level. Elevated carcinoembryonic antigen levels may be present in 50-70% of cases of pancreatic cancer, and alpha-fetoprotein (AFP) levels are elevated in as many as 60% cases of hepatocellular carcinoma.
If the ultrasonographic (US) examination findings show dilated bile ducts but do not provide clues to the site or cause of the obstruction, magnetic resonance cholangiopancreatography (MRCP) or abdominal computed tomography (CT) scanning should be performed next.
The technique is portable, quick, and is the technique of choice in pregnant women and in patients with contrast allergies.
The sensitivity increases with the serum bilirubin concentration and the duration of jaundice. These results were equivalent to the figures for ERCP (91% sensitivity and 92% specificity for any abnormality; 93% sensitivity and 94% specificity for malignant diseases). More importantly, MRCP provides valuable staging information because of its ability to help visualize the hepatic parenchyma and surrounding vascular structures in the same examination. HIDA scanning can help determine the clearance of bile across strictures and surgical anastomosis, thus providing a functional assessment of incomplete strictures and surgical anastomosis.
HIDA scan findings suggest complete biliary obstruction if the small intestine is not visualized in 60 minutes.
Under optimal conditions and using a variety of techniques, cytology sampling can provide a cytologic diagnosis in 75% of cholangiocarcinomas and 50% of pancreatic carcinomas. Advanced cytologic techniques such as digital image analysis (DIA) and fluorescence in situ hybridization (FISH) identify chromosomal abnormalities and improve sensitivity while maintaining high specificity.
The sensitivity of FISH could improve the clinical management of patients being evaluated for malignant bile duct strictures by enabling a definitive diagnosis at earlier stages in the clinical evaluation. A plastic brush collects the cytologic specimen from the lining of the bile duct during an ERCP. The endoscopist localizes the ampulla of Vater, which is on the medial wall of the second portion of duodenum; and the entrance to the bile and pancreatic ducts contained within the ampulla of Vater is then cannulated with specialized catheters. ERCP findings are also valuable for helping differentiate malignant from benign biliary obstruction. ERCP findings show areas of irregular stricturing and dilatation (ie, beading) of the intrahepatic and extrahepatic biliary tree. EUS can help detect choledocholithiasis, especially small stones, with a sensitivity of more than 95%. Porta hepatis lymph nodes are particularly well seen with EUS, in contrast to the relative inability of CT scanning to evaluate the porta region.
Contrast material is injected while the needle is slowly withdrawn, until the bile ducts are opacified. This outlines the intrahepatic and extrahepatic bile ducts and delineates the site of stricture and the anatomy of the fistula. In benign lesions the involved segment of the bile duct is surrounded by a collar of fibrosis causing a narrowing of the lumen (see image below). In contrast, in autoimmune disease, the hallmark finding is dense lymphocytic and plasma cell infiltration (see image below).
The choice of operative repair of a bile duct stricture depends on the location of the stricture. The common hepatic duct is not involved, but the confluence of right and left hepatic ducts is intact. A smooth distal tapering of the CBD associated with proximal dilatation greater than 8 mm is present.
The strictures are 1-2 cm long and do not have features suggesting another cause, such as previous biliary surgery or pancreatitis. Long term treatment of biliary stricture due to chronic pancreatitis with a metallic stent. Management of common bile duct stricture caused by chronic pancreatitis with metal mesh self expandable stents. Reduction of morbidity and mortality from biliary complications after liver transplantation. Hepatic artery stenosis in liver transplant recipients: prevalence and cholangiographic appearance of associated biliary complications. Late biliary tract complications after orthotopic liver transplantation: diagnostic and therapeutic role of endoscopic retrograde cholangiopancreatography.
Endoscopic treatment of sphincterotomy-associated distal common bile duct strictures by using sequential insertion of multiple plastic stents. Endoscopic therapy of anastomotic bile duct strictures occurring after liver transplantation. Posttransplant biliary complications in the pre- and post-model for end-stage liver disease era. The utility of CA 19-9 in the diagnoses of cholangiocarcinoma in patients without primary sclerosing cholangitis. Enhanced detection of cholangiocarcinoma with serum trypsinogen-2 in patients with severe bile duct strictures.
Intraductal ultrasound for the evaluation of patients with biliary strictures and no abdominal mass on computed tomography.
Possible biliary disease: diagnostic performance of high-spatial-resolution isotropic 3D T2-weighted MRCP.
A comparison of routine cytology and fluorescence in situ hybridization for the detection of malignant bile duct strictures.
Meta-analysis of cholecystectomy in symptomatic patients with positive hepatobiliary iminodiacetic acid scan results without gallstones.
A double-blind randomized clinical trial evaluating the analgesic efficacy of ketorolac versus butorphanol for patients with suspected biliary colic in the emergency department. Clinical significance of intrahepatic biliary stricture in efficacy of hepatic resection for intrahepatic stones.
Prospective evaluation of advanced molecular markers and imaging techniques in patients with indeterminate bile duct strictures. Improving biliary-enteric drainage in primary sclerosing cholangitis: experience with endoscopic methods.
Value of MR cholangiography in the diagnosis of obstructive diseases of the biliary tree: a study of 58 cases. Cholangioscopy and cholangioscopic forceps biopsy in patients with indeterminate pancreaticobiliary pathology. Long-term results of endoscopic stenting and surgical drainage for biliary stricture due to chronic pancreatitis. Management of benign biliary strictures: biliary enteric anastomosis vs endoscopic stenting. Topics are richly illustrated with more than 40,000 clinical photos, videos, diagrams, and radiographic images. The articles assist in the understanding of the anatomy involved in treating specific conditions and performing procedures. Check mild interactions to serious contraindications for up to 30 drugs, herbals, and supplements at a time.
Plus, more than 600 drug monographs in our drug reference include integrated dosing calculators. Some pancreatic cysts are true cysts (non-inflammatory cysts), that is, they are lined by a special layer of cells that are responsible for secreting fluid into the cysts. For example, pseudocysts that form after an attack of acute pancreatitis contain digestive enzymes such as amylase in high concentrations. A range of diagnostic tools is available to assess patients with suspected cholangiocarcinoma. Intrahepatic tumors, when confined to a single lobe or segment of the liver, without evidence of metastasis, are resected with their surrounding liver lobe or segments, much as any primary liver tumor is addressed.
At California Pacific Medical Center, we have been leaders in hepatobiliary and pancreas surgery since the founding of our liver transplant program in 1988.
It seems, that PSC and cholangiocarcinoma may represent a spectrum of the same disease process. Extensive subepithelial tumor spread beyond the gross tumor margin is also common and longitudinal spread may extend 15 mm to 20 mm proximally and 5 mm to 10 mm distally (16). Percutaneous transhepatic cholangiography (PTC) is the most useful and definitive radiologic test for bile duct cancer (3). Percutaneous needle biopsies and endoscopic brush biopsies have sensitivity of less than 50% and one can not rely on negative results (18).
Assessing liver function relies on standard laboratory tests of hepatic metabolic and synthetic function.
The two most commonly used are the TNM system devised by the AJCC and the modified Bismuth-Corlette classification for HCCA (20, 21), (Tables 1, 2, Figure 1). Data received from the American National Cancer Institute, referring on 1896 cases of extrahepatic bile duct cancers, showed that 28% had localized disease (AJCC stage, I and II) and were potential surgical candidates (2). Factors precluding curative resection in 60 patients with hilar cholangiocarcinoma (MSKCC, 1991-1997).
Preoperative factors that limited attempts at resection were related to pre-existing patient comorbidities.Intraoperative factors limiting attempted resection were primarily related to findings of cirrhosis, additional abdominal pathology, or extensive fibrosis.
Aggressive management of infection and correction of metabolic and coagulation disorders are also necessary. An end-to-side hepaticojejunectomy to a Roux-Y jejural loop is constructed to establish biliary-enteric continuity.
Dissection of extrahepatic biliary tree from underlying vascular structures is facilitated by the early mobilization of gallbladder and division of supraduodenal common bile duct, as well as encircling left and right hepatic ducts with vessel loops (Figure 3). Following a quick assessment of whether one side of the liver is uninvolved, they resect all but the uninvolved portion of the liver.
Most groups are now routinely performing liver resections for HCCA in 50% to 100% of cases.
Reports on experience with OLT showed that resection yielded equivalent or superior overall 5-year survival at all stages of disease and radical resection itself offered the best quality of life (28). Every attempt should be made to internalize drainage so that patients can avoid the inconvenience and morbidity of external drainage (3). Intraductal brachytherapy catheters after loaded with iridium - 192 seeds are used to deliver 2000 cGy at 1em.
These situations include severe liver disease, patients on total parenteral nutrition, hyperbilirubinemia, inadequate fasting, and alcohol and opiate abuse. Both the pancreas and liver connect to the small intestine at the duodenum, adding important substances such as bile, insulin and glucagon to the intestine. When the ampulla of vater is blocked, the digestive juices produced by the pancreas, which include insulin and glucagon, activate within the pancreas instead of in the duodenum. Fewer than 2,000 cases typically are diagnosed in a year in the United States, and that number constitutes only two-tenths of one percent of all diagnosed gastrointestinal malignancies. In some patients, endoscopic retrograde cholangiopancreatography (ERCP) may be needed for definitive diagnosis and has the advantage of providing a therapeutic option as well (see images below). Thus, MRCP provides a viable alternative to ERCP and allows imaging of the biliary tree when ERCP is unsuccessful; however, MRCP does not currently allow any intervention to be performed, such as stone extraction, stent insertion, or biopsy. This is followed by injection of contrast media into the bile and pancreatic ducts under continuous fluoroscopic monitoring, with visualization of the anatomy of the intrahepatic and extrahepatic bile ducts and the pancreatic duct. The success rate of ERCP is decreased in the presence of a Billroth II, Whipple, or Roux-en-Y anatomy. Infectious causes of biliary obstruction can be diagnosed using collected bile samples or brushings. This is accompanied by a variable amount of inflammatory cellular infiltrate comprising a mixture of neutrophils, lymphocytes, plasma cells, and eosinophils.
Strictures involving the common bile duct (CBD) and low common hepatic duct are easier to repair compared with strictures that are more proximal. Customize your Medscape account with the health plans you accept, so that the information you need is saved and ready every time you look up a drug on our site or in the Medscape app. The head of the pancreas is located on the right side of the abdomen adjacent to the duodenum. Other cysts are pseudocysts (inflammatory cysts) and do not contain specialized lining cells. Mucinous cysts contain mucus (a proteinaceous liquid) produced by the mucinous cells that form the inside lining of the cyst.
Treat your body right with these simple nutrition tips on how to deal with with diarrhea, gas, reflux, and more digestive ailments. The biliary tree within the liver is referred to as a€?intrahepatica€? while a€?extrahepatica€? refers to the biliary system outside the liver. These include noninvasive radiological imaging tests such as ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) or a€?invasivea€? tests.Non-InvasiveUltrasound, CT and MRIUltrasound is often the initial study to rule out more common causes of jaundice. For extrahepatic bile duct cancers, if the lesion by preoperative evaluation appears resectable, the patient is prepared for the operating room and often undergoes a diagnostic laparoscopy immediately prior to the planned surgical resection to rule out unresectable tumor not evident by the preoperative evaluation. Itching is the most commonly reported symptom occurring in up to 30% of patients and may precede the development of jaundice. A two-fold increase in postoperative infections complications have been associated with preoperative stenting (9). Another group of patients carrying an increased risk for cholangiocarcinoma are those with cystic disease of the biliary system in the form of choledochal cysts or Caroli´s disease, due to a chronic biliary inflammation associated with those conditions (7).
CT scans are also highly accurate at imaging small lesions and identifying their relationship to the portal triad and hepatic parenchyma.


It outlines the tumor and proximal biliary system and permits external drainage catheters placement above the lesion. However, in both systems potential hepatic artery or portal venous involvement by the tumor and the functional status of the liver are not taken into account. The majority (72%) presented with either lymph node (46%) or distant (24%) metastases (AJCC stage, III and IV) (2).
Following laparoscopic staging, exploration is undertaken via an extended right subcostal or upper midline incision. The afferent limb should be at least 60 cm to prevent reflux cholangitis, which may result to anastomotic stricture. Preoperative placement of catheters (PTCs) facilitate operative dissection at this stage as well.
Schematic presentation indicating the dissection of the extrahepatic biliary tree facilitated by early mobilization of the gallbladder and division of supraduodenal CBO as well as encircling left and right hepatic ducts with vessel loops. The PTCs are pulled out of the liver surface and silastic catheters are guided into correct position. In this picture, sutures of the posterior row are placed and followed with the ones in the anterior row. The jejunal loop is in place, the reconstruction is completed after the resection of HCC, having secured the silastic tubes inside the lumen of the jejunum. When this approach is used, a negative histological margin is achieved in more than 50% of cases, and is associated with a significant increase or trend towards prolonged survival in all studies. This is accomplished by placement of biliary endoprostheses across the obstructing tumor, which provide drainage from above and below. Subsequently those patients may be treated with 5000 cGy of external beam radiation over 5 to 6 weeks (31). The point at which they connect to the intestine, located at the major duodenal papilla halfway along the second half of the duodenum, is called the ampulla of vater.
Another sphincter, the hepatopancreatic sphincter, controls the movement of liquid through the hepatopancreatic ampulla. This type of cancer occurs in both men and women with equal frequency and is treated by removing the cancer as well as part of the affected duodenum.
Elevated alkaline phosphatase levels are accompanied by increases in gamma-glutamyl transpeptidase and 5' nucleotidase, usually disproportionate to serum transaminase levels. The mucosa of the strictured segment usually is atrophic, with areas of squamous metaplasia. Easily compare tier status for drugs in the same class when considering an alternative drug for your patient. The tail is on the left side of the abdomen, and the body lies between the head and the tail.There are two functional parts to the pancreas, referred to as the exocrine and endocrine parts.
Often these pseudocysts contain pancreatic digestive juices because they are connected to the pancreatic ducts.
For individuals with suspected cholangiocarcinoma, evaluation begins with a computed tomography (CT) scan or magnetic resonance imaging (MRI).
If this laparoscopic evaluation shows extensive disease which cannot be surgically removed, the procedure is stopped without making a large surgical incision. Annually, our physicians provide care to some 4,000 hepatobiliary and pancreas patients, both in San Francisco and at our network of out-reach sites in California and Nevada.
Over 95% are epithelial adenocarcinomas, also known as "cholangiocarcinomas”, predominating in the extrahepatic biliary tree.
Jaundice may be absent in the case of an intrahepatic cholangiocarcinoma or an HCCA that involves only the right or left hepatic duct. Advanced tumors obstructing the portal vein can lead to portal hypertension and splenomegaly (3).
Increased morbidity has been associated with preoperative biliary stenting in patients undergoing either biliary duct excisions or pancreaticoduodenectomy for cholangiocarcinomas (10, 11, 12). Biliary tract infection with parasites endemic to Southeastern Asia, such as Clonorchis sinensis and Opisthorchis viverrin, chronic cholelithiasis, hepatolithiasis and previous cholecystectomy are also accounted among the risk factors for the development of these neoplasms (8).
Delayed images after intravenously contrast-enhanced spiral CT scans are the most sensitive, since cholangiocarcinomas tend to return the contrast longer than adjacent normal tissue (3). Intraoperatively, these catheters assist in the identification of the proximal biliary tract and may be replaced by larger silastic transanastomotic stents if necessary (3). Differential diagnosis includes gallbladder cancer, metastatic disease, rare primary neoplasms (eg carcinoid), Mirrizzi´s syndrome, focal biliary sclerosis and other benign conditions.
Cardiopulmonary evaluation includes pulmonary function tests (most important in smokers) and an EKG. Actually, according to Memorial Sloan Kettering Hospital´s experience, portal vein involvement with tumor was the only independent predictor of respectability (22). Recurrent jaundice and cholangitis can result when plastic endoprostheses are occluded, dislodged or migrate. For unresectable upper-third hilar tumors, proximal hepaticojejunostomies are performed using either the proximal segment III (most commonly) or segment V hepatic duct, with minimal operative mortality and one-year patency rate of 80% (10). At present, the use of chemotherapy in the treatment of cholangiocarcinoma should be limited to clinical trials. Pancreatitis refers to inflammation of the pancreas and can be traced to a variety of other causes as well. The majority of the cells of the pancreas produce digestive juices which contain the enzymes necessary for digesting food in the intestine.
Specific CT imaging protocols and MRI sequences optimize visualization of the biliary system and liver.
For patients requiring hospitalization, we have a dedicated critical care liver unit, hospitalists who specialize in hepatobiliary disease, physician assistants, on-call anesthesia staff and a specialized O.R.
If the tumor spreads towards the portal vein, ipsilateral lobar atrophy can develop, in which case an isolated alkaline phosphatace elevation may be the only clinical finding. On the other hand, several authors have reported the usefulness of biliary stenting, which may provide several advantages such as normalization of coagulation function, reduction of circulating endotoxins, improvement of renal and immune function, and the most important one – facilitate the intraoperative identification of the right and left hepatic ducts at the case of the liver (3, 13).
Chronic typhoid carriers and exposure to asbestos, thorium dioxide and nistrosamines are less well-documented risk factors (8). In selected cases, a celiac and superior mesenteric arteriogram with late-phase portography may be useful to assess resectability, although not much utilized nowadays. If resection or operative bypass are not possible, they can also be internalized to the duodenum or, less often, left to external drainage to alleviate the jaundice (3). In the absence of clear evidence of unresectability, all suspected cholangiocarcinomas should be considered for resection (19).
A more invasive cardiac evaluation is indicated on patients with significant comorbidities or poor performance medical status. Placement of titanium clips in the resection area is necessary to help targeting postoperative external beam radiation therapy.
To prevent such complications, self-expanding metallic biliary endoprostheses, such as the Wallstent should be placed. Few small phase II chemotherapy studies have been reported, where a variety of agents (5-fluoronracil, streptojocitin, mizomycin C, methotrexate, carboplatin) have been used as single agents or in combination. Symptoms can include abdominal pain, nausea and pain in the abdomen that spreads to the lower back. The enzymes are secreted into smaller collecting ducts within the pancreas (side branches). It may be localized (Klatskina€™s tumor) versus a diffuse process (cholangiocellular) within the biliary tree.Early manifestations include pruritis (itchiness) and change in urine and stool color. Computer workstations and software-enabled image reconstruction create 3D views of the biliary anatomy and identify the suspected region of the tumor from various angles. Resectable LesionsIf the initial laparoscopic exploration appears favorable or there is low suspicion of extensive disease, the open surgical procedure ensues with planned removal of the tumor. Most classification systems have separated intrahepatic from extrahepatic tumors and the latter have been traditionally subdivided into 3 groups, based on anatomical location (4). As PTCs are pulled out of liver surface, Silastic catheters are guided into correct position (Figure 4). Metallic stents are superior in the palliation of malignant biliary obstruction and have longer duration of patency (6-8 months) (30).
The side branches empty into a larger duct, the main pancreatic duct, which empties into the intestine through the papilla of Vater in the duodenum. Additional information on the local tumor extent (including vascular involvement) and distant metastatic spread is seen on CT or MRI.InvasiveEndoscopic Retrograde Cholangiopancreatography (ERCP)This diagnostic tool has been the mainstay in evaluating suspected cholangiocarcinoma. The primary surgical objectives are to obtain margins free of residual tumor and removal of associated lymph nodes which may also be involved with the tumor. At California Pacific, our focus is on providing experienced, personalized care for all patients. The efficacy of magnetic resonance cholangiopancreatography (MReP) has been already well documented and has largely replaced invasive methods (percutaneous and endoscopic cholangiography), especially among the advocates of limitation of preoperative biliary instrumentation (17). These authors, though, advocated local bile duct excision only as definitive therapy for some cases of HCAA, and they performed a partial hepatectomy in only 7% of cases.
Failure of jaundiceend symptoms to resolve (usually within few days) after adequate drainage usually indicates underlying lobar atrophy due to vascular compromise of the affected lobe. Finally, photodynamic therapy has been under investigation in the palliative management of extrahepatic cholangiocarcinoma (34). During passage through the ducts, bicarbonate is added to the digestive enzymes to make the pancreatic secretion alkaline. Clinical jaundice occurs later and may be accompanied by abdominal pain, fever and weight loss. Surgical resection offers the only opportunity for cure which, unfortunately, remains low with approximately 25-30% five-year survival due to the frequency of recurrent tumor.
Cancer Navigation ServiceOur Cancer Care Navigation Service provides individuals and families assistance with appointment scheduling, patient education and support service referral. Middle third tumors develop in the region between the upper border of the duodenum and extend to the common bile duct. The cells and ducts producing the digestive juices comprise the exocrine part of the pancreas.Just before the main pancreatic duct enters the duodenum, it usually merges with the common bile duct that collects bile (a fluid that helps to digest fat) produced by the liver. A small catheter inserted into the bile duct with contrast media subsequently injected provides radiographic images of the entire biliary system. The surgery itself has a perioperative mortality of 5-10% with major perioperative morbidity of up to 50%.
Lower third or distal tumors arise between the anpulla of Vater and the upper border of the duodenum (4).
For tumors high in the biliary tree (Bismuth-Corlette Types IIIA and IIIB), the surgical resection involves a partial hepatic resection along with resection of the extra-hepatic biliary tree.
Genetic Risk AssessmentThe Cancer Genetic Risk Assessment Program at California Pacific offers individuals with a personal or family history of cholangiocarcinoma the opportunity to learn more about the genetic nature of their disease and whether they may be predisposed to other cancers which they could monitor.
Intrahepatic cholangiocarcinoma are rare neoplasms, comprising 6 to 10% of all cholangiocarcinomas, and they typically present as solitary hepatic masses (5).
The union of these two ducts forms the ampulla of Vater which drains both the bile and pancreatic fluid into the duodenum through the papilla of Vater.Buried within the tissue of the pancreas, primarily in the head, are small collections of cells, termed the Islets of Langerhans. The cholangioscope provides both visual assessment of strictures and a means to perform visual-guided biopsies. The biliary system is then reconstructed as a hepaticojejunostomy to the remaining hepatic lobe. Individuals meet with our genetic counselor during which an evaluation of onea€™s medical and family history is performed, as well as a detailed risk assessment and genetic education. Tumors at the biliary confluence at the hilum of the liver (so-called Klatskin tumors) are the most frequently encountered and hilar cholangiocarcinomas (HCCA) comprise approximately 60% of the total (6). The average operative mortality rate reported by MSKCC is 6%, which compares favorably to the one from John Hopkins University (3.2%), (4, 22).
The cells of the Islets produce several hormones, for example, insulin, glucagon, and somatostatin; that are released into the blood (the islets do not connect with the pancreatic ducts) and travel in the blood to other parts of the body. Recent advances in endoscopic imaging facilitate the differentiation of tumor from benign inflammatory tissue. For tumors low in the biliary tree (Bismuth-Corlette Type I lesions), a pancreatoduodenectomy is required in addition to the bile duct resection to effect a negative margin.
These hormones have effects throughout the body, for example, insulin, which helps to regulate blood sugar levels. Endoscopic Ultrasound (EUS)EUS involves two complementary platforms: extraductal and intraductal.
Mid-duct tumors (Bismuth-Corlette Type II lesions) can be primarily resected without the need for either a hepatic resection or pancreatectomy.In all cases, reconstruction after the resection involves bringing a fashioned limb of intestine up to the residual bile duct to permit biliary drainage into the intestines. A genetic risk assessment may assist in medical management decisions such as aggressive cancer screening and preventive measures.
Cholangiocarcinoma is also the diagnosis in only 5 to 10% of all periampullary tumors with ampullary, duodenal and pancreatic cancers being the rest of them. The hormone-secreting portion of the pancreas - the Islets - is the endocrine part of the pancreas. Extraductal ultrasound uses an ultrasound probe mounted at the tip of an endoscope to provide detailed, real-time ultrasonic images of the extrahepatic biliary system without instrumenting the bile duct directly.
This is often performed over a stent which remains in place for a period of time postoperatively. Evidence in support of an aggressive approach to resectional therapy for HCCA is reflected, though, in the 40% to 60%. Intraductal ultrasound involves the insertion of a catheter-based ultrasound probe into the bile duct. 3-year survival rates reported in most series in which this strategy was pursued (23, 24, 25).
This probe, which can be advanced into the intrahepatic bile ducts, provides highly detailed images of the bile duct wall lining. Occasionally intraluminal brachytherapy is recommended with similar mixed results experienced and reported to date.Unresectable LesionsFor lesions found at surgical laparotomy to be unresectable, the objective changes from removing the entire lesion with negative margins to instead decompressing the obstructed biliary tree. Percutaneous Transhepatic CholangiographyOccasionally, to characterize the extent of a tumor into the liver, a radiopaque dye is injected into the biliary tree by placing a needle through the abdominal wall into the liver. Depending on the type of tumor and its position in the biliary system, the biliary bypass procedure employed would vary. Unfortunately, with nonresectable lesions, limited life expectancy with median survivals of two to 10 months is anticipated.Transplantation Liver transplantation is generally contraindicated in patients with cholangiocarcinomas. However, a very small minority of carefully screened patients found to have surgically unresectable disease may be considered for liver trans-plantation under experimental protocols with a favorable long-term survival benefit.Stent TherapyEndoscopic therapy consists of dilation of the obstructive biliary segment (with rigid or balloon dilation catheters) and metallic biliary stenting. The goal of treatment is to permanently widen the bile duct to a diameter that allows unimpeded bile flow. As only a small percentage of patients are cured with surgery alone, our focus at California Pacific Medical Center is to integrate all available treatments.



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