cholangiocarcinoma, gallbladder cancer, common bile duct, cystic duct, intrahepatic, perihilar

8/13/2019 cholangiocarcinoma, gallbladder cancer, common bile duct, cystic duct, intrahepatic, perihilar

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Overview of the affected part

The gall bladder and the bile duct are part of the digestive system. Together, the esophagus,

stomach, large and small intestine, aided by the liver, gallbladder and pancreas convert the

nutritive components of food into energy and break down the non-nutritive components into

waste to be excreted.

Anatomy and Physiology of the Gallbladder and Bile Ducts

The anatomy of the biliary tree is a little complicated, butit is important to understand. The liver's cells(hepatocytes) excrete bile into canaliculi, which areintercellular spaces between the liver cells. These draininto the right and left hepatic ducts, after which biletravels via the common hepatic and cystic ducts to thegallbladder. The gallbladder, which has a capacity of 50milliliters (about 5 tablespoons), concentrates the bile 10fold by removing water and stores it until a person eats.

At this time, bile is discharged from the gallbladder viathe cystic duct into the common bile duct and then intothe duodenum (the first part of the small intestine),where it begins to dissolve the fat in ingested food.

The liver excretes approximately 500 to 1000 milliliters (50 to 100 tablespoons) of bile eachday. Most (95%) of the bile that has entered the intestines is resorbed in the last part of thesmall intestine (known as the terminal ileum), and returned to the liver for reuse.


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The many functions of bile are best understood by knowing the composition of bile:

1. Bile Salts (cholates, chenodeoxycholate, deoxycholate): these are produced by the liver'sbreakdown of cholesterol. They function in bile as detergents that dissolve dietary fatand allow it to be absorbed. Hence, disruption of bile excretion disrupts the normalabsorption of fat, a process called malabsorption. Patients develop diarrhea because thefat is not absorbed (steatorrhea) , and develop deficiencies of the fat-soluble vitamins(A, D, E, and K).

2. Cholesterol and phospholipids-while only 4% of bile is cholesterol, the secretion ofcholesterol and its metabolites (bile salts) into bile is the body's major route ofelimination of cholesterol. Phospholipids, which are components of cell membranes,enhance the cholesterol solubilizing properties of bile salts. Inefficient excretion ofcholesterol can cause an increased serum cholesterol. This predisposes to vasculardisease (heart attacks, strokes, etc.)

3. Bilirubin-while this comprises only 0.3% of bile, it is responsible for bile's yellow color.Bilirubin is a product of the body's metabolism of hemoglobin, the carrier of oxygen inred blood cells. Disruption of the excretion of this component of bile leads to a yellow

discoloration of the eyes and skin (jaundice).4. Protein and miscellaneous components

Bile production and recirculation is the main excretory function of the liver. Tumors thatobstruct the flow of bile from the liver can also impair other liver functions. Therefore, it isnecessary to understand these other functions to understand the symptoms that these tumorscan cause. These include:

Metabolic functions, such as the maintenance of glucose (blood sugar) levels

Synthetic functions, such as the synthesis of serum proteins such as albumin, bloodclotting (coagulation) factors, and complement (a mediator of inflammatory responses)

Storage functions, such as the storage of sugar (glycogen), fat (triglycerides), iron,copper, and fat soluble vitamins (A, D, E, and K)

Catabolic functions, such as the detoxification of drugs

The biliary system is comprised of the organs and duct system that create, transport, store and

release bile into the duodenum for digestion. Includes the liver, gallbladder and bile ducts

(named the cystic, hepatic, common, and pancreatic duct).


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the bile duct, which is a 4-inch to 5-inch tube that connects the liver and gallbladder to the

small intestine. Within the liver, smaller tubes, similar to capillaries, drain bile from the cells in

the liver into larger and larger branches, ending in a tube called the common bile duct. The bile

duct allows bile, which is made in the liver and stored in the gallbladder, to flow into the small

intestine. Bile is a liquid that helps to both break down fats found in foods and helps the body

get rid of waste material filtered out of the bloodstream by the liver.

http://www.youtube.com/attribution_link?u=%2Fwatch%3Fv%3D8YlPSOkwIRQ%26feature%3

Dshare&a=rK00QKZ11NS18Yzoq3EWOw

VIDEO OF NORMAL BILE FLOW

OVERVIEW ON CANCER

The body is made up of trillions of living cells. Normal body cells grow, divide into new cells,and die in an orderly way. During the early years of a person's life, normal cells divide faster toallow the person to grow. After the person becomes an adult, most cells divide only to replaceworn-out or dying cells or to repair injuries. Cancer begins when cells in a part of the body startto grow out of control. There are many kinds of cancer, but they all start because of out-of-control growth of abnormal cells. Cancer cell growth is different from normal cell growth.Instead of dying, cancer cells continue to grow and form new, abnormal cells. Cancer cells canalso invade (grow into) other tissues, something that normal cells cannot do. Growing out ofcontrol and invading other tissues are what makes a cell a cancer cell. Cells become cancer cellsbecause of damage to DNA. DNA is in every cell and directs all its actions. In a normal cell,when DNA gets damaged the cell either repairs the damage or the cell dies. In cancer cells, thedamaged DNA is not repaired, but the cell doesnt die like it should. Instead, thiscell goes onmaking new cells that the body does not need. These new cells will all have the same damagedDNA as the first cell does.


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People can inherit damaged DNA, but most DNA damage is caused by mistakes that happenwhile the normal cell is reproducing or by something in our environment. Sometimes the causeof the DNA damage is something obvious, like cigarette smoking. But often no clear cause isfound.

In most cases the cancer cells form a tumor. Some cancers, like leukemia, rarely formtumors. Instead, these cancer cells involve the blood and blood-forming organs and circulatethrough other tissues where they grow. Cancer cells often travel to other parts of the body,where they begin to grow and form new tumors that replace normal tissue. This process iscalled metastasis. It happens when the cancer cells get into the bloodstream or lymph vesselsof our body. No matter where a cancer may spread, it is always named for the place where itstarted. For example, breast cancer that has pread to the liver is still called breast cancer, notliver cancer. Likewise, prostate cancer that has spread to the bone is metastatic prostatecancer, not bone cancer. Different types of cancer can behave very differently. For example,lung cancer and breast cancer are very different diseases. They grow at different rates andrespond to different treatments. That is why people with cancer need treatment that is aimed attheir particular kind of cancer.

Not all tumors are cancerous. Tumors that arent cancer are called benign. Benign tumors cancause problemsthey can grow very large and press on healthy organs and tissues. But theycannot grow into (invade) other tissues. Because they cant invade, they also cant spread to

other parts of the body (metastasize). These tumors are almost never life threatening.

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v%3D8LhQllh46yI%26feature%3Dshare

video of a from a healthy cell to cancer cell

Cholangiocarcinoma is a medical term denoting a form of cancer that is composed of mutatedepithelial cells (or cells showing characteristics of epithelial differentiation) that originate in thebile ducts which drain bile from the liver into the small intestine. Other biliary tract cancersinclude pancreatic cancer, gallbladder cancer, and cancer of the ampulla of Vater.

Cholangiocarcinomas (CCCs) are malignancies of the biliary duct system that may originate inthe liver and extrahepatic bile ducts, which terminate at the ampulla of Vater CCCs areencountered in 3 geographic regions: intrahepatic, extrahepatic (ie, perihilar), and distalextrahepatic. Perihilar tumors are the most common CCCs, and intrahepatic tumors are theleast common. Perihilar tumors, also called Klatskin tumors (after Klatskin's description of them

in 1965), occur at the bifurcation of right and left hepatic ducts. Distal extrahepatic tumors arelocated from the upper border of the pancreas to the ampulla. More than 95% of these tumorsare ductal adenocarcinomas; many patients present with unresectable or metastatic disease.

Cholangiocarcinoma is a relatively rare neoplasm that is classified as an adenocarcinoma (a

cancer that forms glands or secretes significant amounts of mucins). It has an annual incidence

rate of 12 cases per 100,000 in the Western world, but rates of cholangiocarcinoma have been

rising worldwide over the past several decades.


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These tumors can be slow-growing tumors that spread locally via the lymphatic system, as wellas involve local structures such as the blood vessels feeding the liver.

Causes

Cancerous tumors of the bile ducts are usually slow-growing and do not spread (metastasize)

quickly. However, many of these tumors are already advanced by the time they are found.

A cholangiocarcinoma may start anywhere along the bile ducts. These tumors block off the bile

ducts.

They affect both men and women. Most patients are older than 65.

Risks for this condition include:

Bile duct (choledochal) cysts

Chronic biliary and liver inflammation

History of infection with the parasitic worm, liver flukes

Primary sclerosing cholangitis

Ulcerative colitis


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The etiology of most bile duct cancers remains undetermined. Currently,gallstones are not

believed to increase the risk of cholangiocarcinoma. Chronic viralhepatitis andcirrhosis also do

not appear to be risk factors.

Infections

o

In Southeast Asia, chronic infections with liver flukes, Clonorchis sinensis,andOpisthorchis viverrinihave been causally related to cholangiocarcinoma.

o Other parasites, such asAscaris lumbricoides,have been implicated in the

pathogenesis of cholangiocarcinoma.

o Observations have raised the possibility that bacterial infections with

Helicobacterspecies may play an etiologic role in biliary cancer.[9]

Inflammatory bowel disease

o A strong relationship exists between cholangiocarcinoma and primary sclerosingcholangitis. Cholangiocarcinoma generally develops in patients with long-

standing ulcerative colitis and primary sclerosing cholangitis.[10]

o The lifetime risk of developing this cancer in the setting of primary sclerosing

cholangitis is 10-20%. At increased risk are patients with ulcerative colitiswithout symptomatic primary sclerosing cholangitis and a small subset of patients

withCrohn disease. Chemical exposures

o Certain chemical exposures have been implicated in the development of bile duct

cancers, primarily in workers in the aircraft, rubber, and wood-finishing

industries.

o Cholangiocarcinoma occasionally has developed years after administration of the

radiopaque medium thorium dioxide (ie, thorotrast).

Congenital diseases of the biliary tree, including choledochal cysts andCaroli disease,

have been associated with cholangiocarcinoma.

Other conditions rarely associated with cholangiocarcinoma include bile duct adenomas,

biliary papillomatosis, and alpha1 -antitrypsin deficiency.

Symptoms

Chills

Clay-colored stools

Fever

Itching

Loss of appetite

Pain in the upper right abdomen that may radiate to the back

Weight loss

Yellowing of the skin (jaundice)
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Symptoms of Gallbladder and Bile Duct Cancer

Patients with bile duct cancer most often become symptomatic when the cancer obstructs (blocks)the drainage of bile. Because bile cannot be excreted into the bowel, the bilirubin pigmentsaccumulate in the blood, causing jaundice (yellowing of the skin and the whites of the eyes) in 90%of patients. The jaundice is usually associated with itching of the skin (also called "pruritus"). Thebody compensates partially and excretes some of this bilirubin via the urine, so patients may have

dark (cola colored) urine. Because bile cannot reach the intestine, the patient's stools become white(clay colored).


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Other symptoms result from inflammation secondary to tumor obstruction. Patients with gallbladdercancer may have pain in the right upper portion of the abdomen. This pain is a result of inflammationof the gallbladder (cholecystitis) due to blockage of the cystic duct. In fact, approximately 1% ofpatients who undergo cholecystectomy (surgical removal of the gallbladder) for suspectedcholecystitis prove to have unsuspected gallbladder carcinoma. Distal bile duct tumors near theampulla of Vater, the point at which the bile drains into the bowel, obstruct the pancreatic duct and

lead to inflammation of the pancreas (pancreatitis).

Symptoms of cholangiocarcinoma include jaundice, clay-colored stools, bilirubinuria (dark

urine), pruritus, weight loss, and abdominal pain.

Jaundice is the most common manifestation of bile duct cancer and, in general, is best detected

in direct sunlight. The obstruction and subsequent cholestasis tend to occur early if the tumor is

located in the common bile duct or common hepatic duct. Jaundice often occurs later in perihilar

or intrahepatic tumors and is often a marker of advanced disease. The excess of conjugatedbilirubin is associated with bilirubinuria and acholic stools.

Pruritus usually is preceded by jaundice, but itching may be the initial symptom ofcholangiocarcinoma. Pruritus may be related to circulating bile acids.

Weight loss is a variable finding and may be present in one third of patients at the time of

diagnosis.

Abdominal pain is relatively common in advanced disease and often is described as a dull

ache in the right upper quadrant.

Statistics

Primary cholangiocarcinoma is a rare disease. It is estimated that more than 2,500 new cases arediagnosed each year in the United States. However, the incidence of cholangiocarcinoma is

increasing, mostly due to rising rates of intrahepatic cholangiocarcinoma that occurs in the liver.

The reason for this increase is not known. It may be due to doctors having better tests todiagnose this type of cancer more accurately. Previously, they may have been thought to be a

different sort of cancer. In some parts of the world, a parasite called liver flukes can infect the

bile duct and cause cancer to form. Liver flukes are very common in Asia and the Middle East,and therefore cholangiocarcinoma is more common in these regions. Gall stones and

inflammatory conditions of the gastrointestinal (GI) tract, such as ulcerative colitis or an

associated condition called sclerosing cholangitis, increase the risk of cholangiocarcinoma.

The five-year relative survival rate (the percentage of patients who survive at least five yearsafter the cancer is detected, excluding those who die from other diseases) for people diagnosed

with early-stage cholangiocarcinoma is about 30%. However, only about 20% ofcholangiocarcinoma is found at an early stage. The five-year relative survival rate decreases if

cancer has spread at the time of diagnosis.

Cancer survival statistics should be interpreted with caution. These estimates are based on data

from thousands of cases of this type of cancer in the United States and may not apply to a single


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person. It is not possible to tell a person how long he or she will live with cholangiocarcinoma.

Because the survival statistics are measured in five-year (or sometimes one-year) intervals, they

may not represent advances made in the treatment or diagnosis of this cancer.

Epidemiology

Cholangiocarcinoma is anadenocarcinoma of thebiliary tract,[85]

along withpancreatic cancer

(which occurs about 20 times more frequently),[86]

gall bladder cancer (which occurs twice as

often), and cancer of theampulla of Vater.Treatments and clinical trials for pancreatic cancer,being far more prevalent, are often taken as a starting point for managing cholangiocarcinoma,

even though the biologies are different enough that chemotherapies can put pancreatic cancer

into permanent remission whereas there are no reports in the literature of long-term survival due

to chemotherapy or radiation applied to aninoperable cholangiocarcinoma case.

Cholangiocarcinoma is a relatively rare form of

cancer; each year, approximately 2,000 to

3,000 new cases are diagnosed in the UnitedStates, translating into an annualincidence of

12 cases per 100,000 people.[1]

Autopsy series

have reported aprevalence of 0.01% to

0.46%.[88][89]

There is a higher prevalence ofcholangiocarcinoma in Asia, which has been

attributed to endemic chronic parasitic

infestation. The incidence of

cholangiocarcinoma increases with age, andthe disease is slightly more common in men

than in women (possibly due to the higher rate

ofprimary sclerosing cholangitis,a major riskfactor, in men).

[38]The prevalence of cholangiocarcinoma in patients with primary sclerosing

cholangitis may be as high as 30%, based on autopsy studies.[12]

Multiple studies have documented a steady increase in the incidence of intrahepatic

cholangiocarcinoma over the past several decades; increases have been seen inNorth America,

Europe,Asia,andAustralia.[90]

The reasons for the increasing occurrence of cholangiocarcinoma

are unclear; improved diagnostic methods may be partially responsible, but the prevalence of

potential risk factors for cholangiocarcinoma, such asHIV infection,has also been increasingduring this time frame.

[19]

Epidemiology

Frequency

CountryIC

(men/women)

EC

(men/women)

U.S.A. 0.60 / 0.43 0.70 / 0.87

Japan 0.23 / 0.10 5.87 / 5.20

Australia 0.70 / 0.53 0.90 / 1.23

England/Wales 0.83 / 0.63 0.43 / 0.60

Scotland 1.17 / 1.00 0.60 / 0.73

France 0.27 / 0.20 1.20 / 1.37

Italy 0.13 / 0.13 2.10 / 2.60

Age-standardized mortality ratesfrom

intrahepatic (IC) and extrahepatic (EC)

cholangiocarcinoma for men and women, by

country. Source: Khan et al., 2002.[87]
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United States

Each year, approximately 2500 cases of CCC occur, compared to 5000 cases ofgallbladdercancerand 15,000 cases of hepatocellular cancer. Average incidence is 1 case per 100,000

persons per year.

A study by Singal et al found that the frequency of intrahepatic cholangiocarcinoma has

increased over time and is most commonly noted in females older than 60 years.[7]

International

Incidence in most Western countries ranges from 2 to 6 cases per 100,000 people per year. The

highest annual incidences are in Japan, at 5.5 cases per 100,000 people, and in Israel, at 7.3 cases

per 100,000 people.

Mortality/Morbidity

Despite aggressive anticancer therapy and interventional supportive care (ie, wall stents orpercutaneous biliary drainage), median survival rate is low, since most patients (90%) are not

eligible for curative resection. The overall survival is approximately 6 months.

Race

Native Americans have the highest annual incidence in North America, at 6.5 cases per 100,000people. This rate is about 6 times higher than that in nonNative American populations. The

high prevalence of cholangiocarcinoma in people of Asian descent is attributable to endemic

chronic parasitic infestation.

Sex

The male-to-female ratio for cholangiocarcinoma is 1:2.5 in patients in their 60s and 70s and1:15 in patients younger than 40 years. According to theAmerican Cancer Society,the number

of new cases of liver and intrahepatic bile duct cancer in 2007 is estimated to be 13,650 for men

and 5,510 for women, with estimated mortality of 11,280 and 5,500, respectively. The estimatednumber of new cases of gallbladder and other biliary cancers (extrahepatic cholangiocarcinoma)

are 4,380 for men and 4,870 for women, with estimated mortality rates of 1,260 and 1,990,

respectively.[8]

Age

Highest prevalence rate occurs in males and females in their 60s and 70s.

Staging
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Staging is a way of describing a cancer, such as where it is located, if or where it has spread, and

if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to

determine the cancer's stage, so staging may not be complete until all the tests are finished.Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict

a patient's prognosis (chance of recovery). There are different stage descriptions for different

types of cancer.

One tool that doctors use to describe the stage is the TNM system. This system uses three criteria

to judge the stage of the cancer: the tumor itself, the lymph nodes around the tumor, and if thetumor has spread to the rest of the body. The results are combined to determine the stage of

cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through

four). The stage provides a common way of describing the cancer so doctors can work together

to plan the best treatments.

TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three

factors to determine the stage of cancer:

How large is the primary tumor and where is it located? (Tumor, T)

Has the tumor spread to the lymph nodes? (Node, N)

Has the cancer metastasized (spread) to other parts of the body? (Metastasis, M)

Intrahepatic cholangiocarcinoma (cancer that occurs in the bile duct within the liver) is staged

using the same system as liver cancer. The staging of both intrahepatic and extrahepaticcholangiocarcinoma is below.

Intrahepatic cholangiocarcinoma staging

Tumor. Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the

stage of intrahepatic cholangiocarcinoma. If there is more than one tumor, the lowercase letter"m" (multiple) is added to the "T" category. Specific tumor stage information is listed below:

TX: The primary tumor cannot be assessed.

T0: There is no evidence of a primary tumor.

T1:The tumor is only a single tumor. It does not involve adjacent blood vessels.

T2:Either of these conditions:

Any tumor that involves adjacent blood vessels is present.

Multiple tumors, none larger than 5 centimeters (cm), are present.


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More than one tumor larger than 5 cm is present.

The tumor involves the major veins within the liver.


The tumor has spread to the organs near the liver (except the gallbladder).

The tumor is present with perforation of the visceral peritoneum (layer of tissuethat lines the abdomen).

Node.The "N" in the TNM staging abbreviation means node. Lymph nodes are tiny, bean-

shaped organs located all over the body that normally help fight infections and cancer as part ofthe body's immune system. Each type of tumor drains into lymph nodes nearby called regional

lymph nodes.

NX:The regional lymph nodes cannot be assessed.

N0:Cancer has not spread to the regional lymph nodes.

N1:The cancer has spread to the regional lymph nodes.

Distant metastasis.The "M" in the TNM system indicates whether the cancer has spread to

other parts of the body.

MX:The tumor cannot be assessed.

M0:The cancer has not metastasized.

M1:There is metastasis to another part of the body.

Cancer stage grouping for intrahepatic cholangiocarcinoma

Doctors assign the stage of the cancer by combining the T, N, and M classifications.

Stage I:This is the earliest stage of intrahepatic cholangiocarcinoma. The tumor has not spread

to the blood vessels, lymph nodes, or other parts of the body (T1, N0, M0).

Stage II:The tumor involves nearby blood vessels, but has not spread to the regional lymph

nodes or other parts of the body (T2, N0, M0).


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Stage IIIA:The cancer has not spread beyond the liver, but the area of the cancer is larger than

stage I or II, and it often has invaded nearby blood vessels (T3, N0, M0).

Stage IIIB:The cancer has spread to organs near the liver, but has not spread to nearby lymph

nodes or other parts of the body (T4, N0, M0).

Stage IIIC:Any tumor that has spread to the regional lymph nodes, but not to other parts of the

body (any T, N1, M0).

Stage IV:Any tumor that has spread to other parts of the body (any T, any N, M1).

Extrahepatic cholangiocarcinoma staging

Tumor. Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the

stage of extrahepatic cholangiocarcinoma. If there is more than one tumor, the lowercase letter"m" (multiple) is added to the "T" category. Specific tumor stage information is listed below:

TX:The primary tumor cannot be assessed.

T0:There is no evidence of a primary tumor.

Tis: Refers to carcinoma (cancer) in situ. (Cancer is only in a single layer of cells lining the bile

duct and not invading other parts of the bile duct wall.)

T1:The tumor is confined to the bile duct.

T2:The tumor has spread beyond the wall of the bile duct.

T3:The tumor has spread to the liver, gallbladder, pancreas, and/or an unilateral branch (a single

side) of the veins and/or arteries within the liver.

T4:The tumor has spread bilaterally (both sides) to the veins or arteries within the liver and/or

adjacent structures, such as the colon, stomach, duodenum, or abdominal wall.

Node.The "N" in the TNM staging abbreviation means node. Lymph nodes are tiny, bean-

shaped organs located all over the body that normally help fight infections and cancer as part of

the body's immune system. Each type of tumor drains into lymph nodes nearby called regionallymph nodes.

NX:The regional lymph nodes cannot be assessed.

N0:Cancer has not spread to the regional lymph nodes.

N1:The cancer has spread to the regional lymph nodes.


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Distant metastasis. The "M" in the TNM system indicates whether the cancer has spread to

other parts of the body.

MX:The tumor cannot be assessed.

M0:The cancer has not metastasized.

M1:There is metastasis to another part of the body.

Cancer stage grouping for extrahepatic cholangiocarcinoma

Doctors assign the stage of the cancer by combining the T, N, and M classifications.

Stage 0:This is the earliest stage of extrahepatic cholangiocarcinoma. The cancer is only found

in one layer of cells, and has not spread (Tis, N0, M0).

Stage IA:The tumor is confined to the bile duct (T1, N0, M0).

Stage IB:The tumor has spread beyond the wall of the bile duct, but has not spread to theregional lymph nodes or other parts of the body (T2, N0, M0).

Stage IIA: The tumor involves adjacent organs, such as the liver, gallbladder, pancreas, and/orunilateral branches of adjacent blood vessels, but has still not spread to the regional lymph nodes

or other parts of the body (T3, N0, M0).

Stage IIB:The tumor may or may not involve unilateral branches of adjacent blood vesselsand/or the tumor involves nearby organs, such as the liver, gallbladder, and pancreas. The cancer

has spread to nearby lymph nodes, but has not spread to other parts of the body (T1 or T2 or T3,

N1, M0).

Stage III: The tumor has spread bilaterally to adjacent blood vessels and/or adjacent structures,and may or may not have spread to the regional lymph nodes, but has not spread to other parts of

the body (T4, any N, M0).

Stage IV: Any tumor that has spread to other parts of the body (any T, any N, M1).

Exams and Tests

Your health care provider will perform a physical exam. Tests will be done to check for a tumor or

blockage in the bile duct. These may include:

Abdominal CT scan

Abdominal ultrasound


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CT scan-directed biopsy

Cytology of samples from the bile duct

Endoscopic retrograde cholangiopancreatography (ERCP)

Magnetic resonance cholangiopancreatography (MRCP)

Percutaneous transhepatic cholangiogram (PTCA)

Blood tests that may be done include:

Liver function tests (especially alkaline phosphatase or bilirubin levels)

How is cholangiocarcinoma diagnosed?

When you see your physician you will have a routine examination. Your doctor will ask you

questions about your general health and your family history of cancer and liver disease. You willalso be asked about your lifestyle and habits, including drinking and smoking.

Your physician may order the following tests:

Blood work.Blood tests may include a complete blood count, hematocrit, platelet count,

liver function tests, Carcinoembriogenic antigen (CEA) and Carbohydrate antigen 19-9(CA19-9), which may be elevated in patients with bile duct cancer.

Abdominal ultrasound.This test helps the doctor see the tumor.

CT scan.This test identifies the tumor(s) and pinpoints their size and location in theliver, as well as their relation to the vascular / biliary structures. It also helps the doctor to

determine the overall health of the liver.


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Comparison of radiographic images showing cholangiocarcinoma; A, computed tomography

(CT) image; B, cholangiogram (ERCP) image. Arrows designate the tumor.

MRI.This test identifies the tumor(s) and pinpoints their size and location in the liver, aswell as their relation to the vascular / biliary structures. It also helps the doctor to

determine the overall health of the liver. A doctor will determine whether to do a CTscan, an MRI or both.

Endoscopy.Endoscopy is visual instrument that allows your physician to see the inside

of the esophagus, stomach and beginning of the lower intestine without surgery.

Room setup and patient positioning for endoscopic retrograde cholangiopancreatography

(ERCP)

Endoscopic Retrograde Cholangiopancreatography (ERCP). Endoscopic retrograde

cholangiopancreatography is an endoscopic procedure that involves the use of fiberoptic

endoscopes. You will be lightly sedated and your doctor will insert an endoscope throughthe mouth, down the esophagus, and into the stomach and small bowel. A smaller tube or

catheter is passed through the endoscope and into the bile ducts. Dye is injected into the


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ducts, and the doctor takes X-rays that can show whether a tumor is present in the bile

ducts.

A, B, Position of the endoscope in the duodenum during ERCP

Percutaneous (through the skin) Transhepatic (through the liver)Cholangiogram. In this procedure, a thin needle is inserted through the skin and into the

bile ducts. A dye is injected through the needle so that a contrast image will show up on

X-rays. By looking at the X-rays, the doctor may be able to see whether there is a tumorin the bile ducts.


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A, technique of transhepatic percutaneous cholangiography; B, corresponding percutaneous

cholangiograph (after catheter is introduced)

What is the treatment for cholangiocarcinoma?

Treatment for bile duct cancer may include a combination of the following:

Surgeryto remove the bile duct, and sometimes part of the liver Chemotherapy

Radiation

Stent placement. A stent is a tube that allows the bile, which is made by the liver, to drain

more easily into the intestine if a tumor is blocking the bile duct.
http://www.hopkinsmedicine.org/liver_tumor_center/treatments/surgery_remove_tumor.htmlhttp://www.hopkinsmedicine.org/liver_tumor_center/treatments/surgery_remove_tumor.htmlhttp://www.hopkinsmedicine.org/liver_tumor_center/treatments/chemotherapy.htmlhttp://www.hopkinsmedicine.org/liver_tumor_center/treatments/chemotherapy.htmlhttp://www.hopkinsmedicine.org/liver_tumor_center/treatments/ablative_techniques/index.htmlhttp://www.hopkinsmedicine.org/liver_tumor_center/treatments/ablative_techniques/index.htmlhttp://www.hopkinsmedicine.org/liver_tumor_center/treatments/ablative_techniques/index.htmlhttp://www.hopkinsmedicine.org/liver_tumor_center/treatments/chemotherapy.htmlhttp://www.hopkinsmedicine.org/liver_tumor_center/treatments/surgery_remove_tumor.html


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A, point of percutaneous approach on a patient; B, right and left percutaneous transhepaticbiliary drainage catheters restore patency around a hilar tumor

VIDEO

Billiary Drain: http://www.youtube.com/watch?v=dWyHMm... The subject of this short

video is the Percutaneous Transhepatic Biliary Drain. The tube and the medical ...

Surgical Therapy

Removing biliary tract tumors surgically is the treatment of choice in cholangiocarcinoma as it is

the only therapeutic option that offers the potential for cure. Surgical approaches have becomeincreasingly sophisticated over the last decade. It has become apparent that curative treatment

depends on expertise that often involves the removal of a major section of the liver. The

objective is complete removal of the bile duct and tumor.


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The treatment of cholangiocarcinoma depends on the size and location of the tumor, whether the

cancer has spread, and the persons overall health. In many cases, a team of doctors will workwith the patient to determine the best treatment plan. The main treatment for bile duct cancer issurgery. Radiation therapy and chemotherapy may be used if the cancer cannot be entirely

removed with surgery and in cases where the edges of the tissues removed at the operation show

cancer cells (also called a positive margin). Both stage III and stage IV cancers cannot becompletely removed surgically.

Surgery

Due to the location and sensitivity of the bile duct area, surgery for cholangiocarcinoma can be

difficult. If the cancer is near the liver, part of the liver will be removed, along with the bile duct,

gallbladder, and sometimes part of the pancreas and small intestine. If the cancer is near thepancreas, the surgeon may need to remove some or all of the pancreas and some small intestine.

In order to maintain appropriate flow of bile, the remaining part of the bile duct has to be

connected to the small intestine. About 5% to 10% of people do not survive this complicatedoperation; others (25% to 45%) experience serious complications, such as bleeding, infection, or

leaking of bile or pancreatic juices. In some cases, surgeons cannot completely remove the

tumor. Therefore, the surgeon bypasses the blocked area by connecting part of the bile duct


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before the blockage with a part of the small intestine beyond the blockage. The surgeon may

insert a stent (a plastic or metal tube) into the bile duct to keep it open.

If the doctors think that the tumor cannot be removed by surgery, a plastic or metal stent can be

passed through the blockage either during the ERCP procedure or during a procedure similar to

PTC. Although these procedures do not remove the tumor, they relieve its effects and peopleoften experience long periods of time when all of their symptoms disappear and quality of life is

much better. For both these procedures, the doctor will try to insert the stent internally, so the

person will not be aware of its presence. Sometimes, this is not possible, and a tube will bepassed through the liver to redirect the bile externally into a bag that will need regular changing.

Some doctors suggest that in these situations people receive long-term antibiotics to guard

against infection.

Liver transplantation

Complete removal of the liver and bile ducts followed by transplantation of a donor liver has

been used to treat this type of cancer. This is a major procedure. Medical professionals are not inagreement about its usefulness for cholangiocarcinoma.

Radiation therapy

Radiation therapy uses high-energy x-rays to damage cancer cells, and several treatments may be

needed. External-beam radiation (radiation given from a machine outside the body) is most often

used to treat people with cholangiocarcinoma. Occasionally, internal radiation therapy may beused for cholangiocarcinomas; this involves inserting small radioactive pellets near or in the

cancer by way of a tube. Side effects of radiation therapy include skin irritation, nausea, and

fatigue. Radiation therapy can be used for treatment or to control symptoms and pain in

advanced disease.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy may be used before surgery

to shrink the tumor or when surgery is not an option. In some cases, chemotherapy can shrink thetumor, but it has not yet been proven that this improves the quality of life or prolongs life. Drugs

that have been used for cholangiocarcinoma include fluorouracil (5-FU, Adrucil, Efudex),

cisplatin (Platinol), doxorubicin (Adriamycin, Rubex), and gemcitabine (Gemzar). Side effects

depend on the drug and the dosage. Common side effects include nausea and vomiting, loss ofappetite, diarrhea, fatigue, low blood count (which increases the body's susceptibility to

infection), bleeding or bruising after minor cuts or injuries, numbness and tingling in the hands

or feet, headaches, hair loss, and darkening of the skin and fingernails. Side effects usually go

away when treatment is complete.

Efforts to improve chemotherapy by investigating new drugs or new combinations of drugs arebeing made through clinical trials. This is often how patients with cholangiocarcinoma receive

chemotherapy.


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The medications used to treat cancer are continually being evaluated. Talking with your doctor is

often the best way to learn about the medications youve been prescribed, their purpose, and their

potential side effects or interactions with other medications.

http://www.hopkinsmedicine.org/gastroenterology_hepatology/_pdfs/pancreas_biliary_trac

t/cholangiocarcinoma.pdf for download of other diagnostic images
http://www.hopkinsmedicine.org/gastroenterology_hepatology/_pdfs/pancreas_biliary_tract/cholangiocarcinoma.pdfhttp://www.hopkinsmedicine.org/gastroenterology_hepatology/_pdfs/pancreas_biliary_tract/cholangiocarcinoma.pdfhttp://www.hopkinsmedicine.org/gastroenterology_hepatology/_pdfs/pancreas_biliary_tract/cholangiocarcinoma.pdfhttp://www.hopkinsmedicine.org/gastroenterology_hepatology/_pdfs/pancreas_biliary_tract/cholangiocarcinoma.pdfhttp://www.hopkinsmedicine.org/gastroenterology_hepatology/_pdfs/pancreas_biliary_tract/cholangiocarcinoma.pdf

cholangiocarcinoma, gallbladder cancer, common bile duct, cystic duct, intrahepatic, perihilar

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