Bile duct cancer develops in the bile duct. The disease may occur at any age, but people over 60 years of age are at increased risk. It is important to consult the doctor if the patient experiences any symptoms, as bile duct carcinoma diagnosed at an advanced stage is difficult to treat.
Bile duct cancer, or bile duct carcinoma, initiates in the bile duct. Bile ducts are tube-like structures that carry the bile from the liver and gallbladder into the digestive system (small intestine). There are several types of bile duct tumors. However, not all of them are cancerous. Bile duct adenomas and bile duct hamartomas are non-cancerous. Almost all bile duct cancers are cholangiocarcinomas, and thus, the term can be used as a synonym for this cancer type. Cholangiocarcinoma is a type of adenocarcinoma, and it begins in the glandular cells. These glandular cells line the inner layer of the bile duct.
The incidence and mortality of bile duct carcinoma vary depending on geographical regions, gender, and types of bile duct carcinoma. A few studies have reported the highest incidence in Chile and the lowest in Vietnam. Most countries have had a trend of increasing incidence rates in recent times. Patients aged 75 years or older have 5 to 10 times higher mortality rates compared to the overall mortality rate. Males have a higher incidence and mortality rate for extrahepatic cholangiocarcinoma, ampulla of Vater cancer, and intrahepatic cholangiocarcinoma.
Depending on the site of origin, bile duct carcinoma is classified into various types. Following are the various bile duct cancer types:
Intrahepatic cholangiocarcinoma is the second most common type of cancer arising from the liver, after hepatocellular carcinoma. ICC accounts for almost 10% of all bile duct cancer types. Only about 15% of the ICC tumors are resectable, and the patients have a median survival period of three years.
ECC develops outside the liver. ECC may develop at the perihilar and distal anatomical sites in the bile duct. There is a global increase in the incidence of ECC. Surgical resection is the most common treatment for ECC during the early stages. However, when presented as a metastatic or unresectable disease, ECC has a high risk of recurrence that restricts optimal survival.
The left and right bile ducts leave the liver and combine to form the common hepatic duct. Perihilar cancer occurs in the bile duct in the area where these two ducts leave the liver. The condition is also known as a Klatskin tumor, as Gerald Klatskin initially described it. It is usually diagnosed in the advanced stage and has a poor prognosis. Currently, the primary management option for perihilar bile duct carcinoma is a complete hepatectomy.
The ducts from the gallbladder and the liver combine to form a common bile duct. The common bile duct delivers the bile into the small intestine. Distal cholangiocarcinoma occurs in the area of connection between the gallbladder and liver ducts. Due to its anatomical location and inherent aggressiveness, patients have a poor prognosis. Surgical resection is the primary management option for this condition. The surgeon may also perform pancreaticoduodenectomy or hepatopancreaticoduodenectomy.
Hilar cholangiocarcinoma is a rare condition and is more common in people over 60 years of age. Patients with this condition usually have a poor prognosis. It comprises over 60% of cholangiocarcinoma cases.
Staging is an important aspect of bile duct carcinoma management. It helps doctors understand the extent of the disease and, accordingly, create a treatment plan. The following are the different stages of bile duct cancer (intrahepatic cholangiocarcinoma):
Stage 0 bile duct cancer is also known as carcinoma in situ. In this stage, the abnormal cells are in the innermost intrahepatic duct lining. These cells are not cancerous; however, they can potentially transform into malignant cells and spread to surrounding healthy tissues.
Stage 1 bile duct cancer is divided into stages 1A and 1B. The tumor in stage 1A bile duct carcinoma is limited to intrahepatic bile duct and is of size ≤5 cm. The tumor in stage 1B bile duct carcinoma is limited to the intrahepatic bile duct and has a size >5 cm.
Stage 2 bile duct cancer is characterized by the presence of a tumor spread into the intrahepatic bile duct wall or into a blood vessel, or by the presence of more than one tumor in the intrahepatic bile duct that may have spread into a blood vessel.
Stage 3 bile duct cancer is divided into stages 3A and 3B. In stage 3A bile duct carcinoma, the tumor has spread to the outer liver lining. In stage 3B bile cancer, the cancer has spread into the surrounding tissues and organs of the liver, such as the diaphragm, abdominal wall, common bile duct, colon, duodenum, stomach, and a part of the vena cava. The cancer in stage 3B would have also spread to the nearby lymph nodes. However, the cancer has not spread to distant organs.
Stage four bile duct cancer refers to the most advanced stage. This stage is characterized by the spread of the disease to distant organs, such as the lungs, bones, and distant lymph nodes.
Patients with bile duct cancer experience various symptoms. These bile duct cancer symptoms may be similar to those of various other less serious conditions. It is important to consult the doctor if one starts experiencing any signs and symptoms of bile duct cancer. Having liver and gallbladder diseases is considered to be one of the possible bile duct cancer causes.
Anyone can get bile duct cancer, irrespective of age, gender, and geographical location. However, some population groups are at a relatively higher risk of developing this cancer type compared to other groups. Bile duct cancer mainly occurs in people over the age of 60.
Patients suffering from several underlying medical conditions, such as primary sclerosing cholangitis, obesity, inflammatory bowel disease, non-alcoholic fatty liver disease, bile duct stones, liver fluke infections, and hepatitis B and C infections, also have a high risk of developing bile duct cancer. People who consume alcohol excessively and have a family history of bile duct cancer are also more likely to be diagnosed with bile duct cancer.
Bile duct cancer presents with multiple symptoms. It is important to see a doctor when one experiences persistent symptoms that do not go away even with medications. The following are the commonly observed bile duct cancer symptoms:
It is one of the most common signs of bile duct cancer. When there is an obstruction in the bile flow due to a bile duct tumor, the bile flows back into the tissues and the bloodstream, resulting in yellowing of the skin and eyes.
When the bile accumulates in the skin tissues, the level of bilirubin increases. High bilirubin levels in the skin tissues result in itching. Removing obstruction in the bile ducts may help manage itching. Some patients have itching from the substances secreted by the cancer cells. Cancer-induced itching can be managed by treating cancer.
Unexplained weight loss may be an early sign of bile duct cancer. There are several reasons for a patient to have unintentional weight loss during bile duct cancer. First, patients with bile duct cancer have a low appetite, leading to reduced calorie intake. Further, the absence or reduced level of bile in the small intestine (because of bile duct obstruction) may interfere with fat absorption.
Loss of appetite may also be a cholangiocarcinoma cancer symptom. It may occur both before and after the diagnosis. Before the diagnosis, patients do not feel hungry due to interference with liver-assisted metabolism and inadequate fat absorption. This interference may lead to difficulty in the absorption of various nutrients. After the cancer diagnosis, poor psychological health plays a role in losing appetite.
Patients with bile duct cancer have high levels of bilirubin, accompanied by fever. Bile duct obstruction due to bile duct cancer may result in infection, leading to fever.
Abdominal pain may also be one of the bile duct cancer symptoms. Patients usually experience pain in the right lower quadrant, below the ribs. Abdominal pain usually occurs in the advanced stage of bile duct cancer when the cancer spreads to surrounding tissues. Several other conditions, such as gallstones, may also cause abdominal pain.
Patients with bile cancer also have alterations in the texture and appearance of their stools. Patients with bile duct cancer have clay-colored or white stools. The stools of such patients are oily due to poor digestion and absorption of fat. The color change is due to the absence of bile in the stools.
There are several reasons for fatigue to present as a symptom of bile duct cancer. Patients experience extensive tiredness during their cancer treatment or when the cancer progresses to an advanced stage. Loss of appetite is one of the reasons for fatigue and weakness. Further, as cancer cells are rapidly growing, they require significant energy, which causes fatigue and weakness in patients. Patients with advanced cancer experience more fatigue as compared to early-stage cancer patients.
Dark urine is also one of the bile duct cancer symptoms. When the bile duct is obstructed, the bile flows back and accumulates in tissues. The excess bilirubin is then excreted in the urine, resulting in dark-colored urine.
Night sweats may be a symptom of bile duct cancer or be due to the treatment of bile duct cancer. Night sweats may suggest that the body is fighting against cancer or infection.
The exact cause of bile duct cancer is yet to be identified. However, certain factors that can increase the risk of bile duct cancer have been identified. These factors may be referred to as risk factors or possible causes. The following are the possible bile duct cancer causes:
Chronic diseases, such as inflammatory bowel disease, non-alcoholic fatty liver disease, diabetes, and obesity, could be the possible bile duct cancer causes.
Patients with hepatitis C and B virus infections are more likely to develop bile duct cancer. This is because untreated hepatitis infections may result in the development of cirrhosis, which is also a cause of bile duct cancer
Chronic liver and bile duct inflammation is one of the most common bile duct cancer causes. Several liver and bile duct diseases may result in chronic inflammation. These include primary sclerosing cholangitis, choledochal cyst disease, bile duct stones, liver fluke infections, and cirrhosis.
The presence of gallstones could also be a potential bile duct cancer cause. It may be due to chronic irritation and inflammation of the bile ducts.
There are several mechanisms through which obesity causes bile duct cancer. First, obesity alters the level of certain hormones, leading to bile duct cancer. Second, obesity also causes various other diseases, such as gallstones, bile duct stones, and non-alcoholic fatty liver disease.
Several diagnosis and treatment options are available for bile duct cancer management. These options help in the overall management of bile duct cancer. The choice of bile duct cancer treatment depends upon several factors, such as stage of diagnosis, health of the patient, age, underlying medical condition, and response to treatment.
Following are some of the methods for bile duct cancer diagnosis:
The patients suspected of having bile duct cancer undergo a detailed examination of their overall health and medical history. The patients are asked about the symptoms, their onset, and their severity.
The medical history of the patients is also evaluated to detect underlying medical conditions, such as irritable bowel syndrome, diabetes, obesity, cirrhosis, and primary sclerosing cholangitis. The doctor may also ask about the family history of the patients, as some cases of bile duct cancer may also be hereditary. If the doctor strongly suspects cancer or other serious conditions, the patients are advised to undergo further tests.
Liver function tests provide detailed information about the overall status of liver health. The tests include the evaluation of various enzymes and other chemicals, such as bilirubin, synthesized by the liver. Patients with bile duct cancer have high levels of bilirubin in the blood. High levels of bilirubin may also be due to several reasons other than cancer.
Liver function tests are one of the laboratory tests for detecting liver status. Other laboratory tests can also be performed to examine the overall health status. These may include blood sugar tests, cholesterol levels, and a complete blood count. The patients may also undergo tumor marker tests to detect the level of CEA and CA 19-9 that may be elevated in patients with cancer. However, these markers may also be elevated in conditions other than cancer.
Ultrasound imaging techniques are used to detect bile duct cancer. It uses ultrasound waves to obtain images. It is usually the first imaging test advised in case the patient experiences symptoms of bile duct cancer, such as abdominal pain or jaundice.
A CT scan may also be recommended for bile duct cancer diagnosis. A CT scan provides important information about the presence of cancer and its spread to the surrounding tissues and lymph nodes. It also assists in the staging of the disease. A CT scan is also used in several techniques for diagnosing bile duct cancer, such as CT angiography and CT-guided biopsy.
It uses magnetic fields and radio waves to provide images of the bile duct and nearby organs for diagnosing bile duct cancer. The technique is also helpful in differentiating benign or non-cancer bile duct tumors from malignant tumors. The doctors may inject a contrast agent, such as gadolinium, to obtain clearer images.
MRCP is also a cholangiocarcinoma diagnostic test that provides detailed images of the bile duct with the assistance of the standard MRI technique. The technique does not involve using a contrast agent or an endoscope, unlike other cholangiograms. If images of the bile ducts are needed, the doctors prefer this technique due to its non-invasive characteristics.
Angiography is often recommended for bile duct cancer diagnosis. It involves the use of a catheter for evaluating the blood vessels present in and around the bile ducts and the liver. During this procedure, a contrast dye is administered with the help of an IV line. The MRI is then taken to detect the presence of any blockage in the blood vessels.
A biopsy is a procedure for diagnosing bile duct cancer and is usually considered a confirmatory test for diagnosis. This procedure involves taking tissue samples and examining them for the presence of cancer cells.
Laparoscopic surgery is performed with the assistance of a laparoscope. The technique is used to determine the presence of tumors in the bile duct and to obtain samples of the abnormal tissue for analysis in the laboratory.
This procedure is performed under the influence of sedative drugs. The doctor may also use local anesthesia to minimize the pain and discomfort. During this procedure, a needle is guided into the bile duct through the abdominal wall to inject the contrast dye. The patient then undergoes an X-ray examination to trace the contrast. Samples of abnormal tissues may also be obtained during PTC.
During this procedure, the doctor guides an endoscope into the initial section of the small intestine. A small catheter from the endoscope is inserted into the common bile duct, through which a contrast dye is injected. The X-ray images are taken to detect the blockage in the bile duct and the pancreatic duct. A tissue sample may also be obtained during ERCP.
It involves the use of an endoscope and ultrasound device to obtain images. A needle is also attached to the endoscope to obtain the tissue samples.
Bile duct cancer treatment often demands a multidisciplinary approach wherein specialists from various disciplines thoroughly study the case before devising an individualized treatment plan. The different bile duct cancer treatment options recommended as part of the treatment plans include:
Bile duct cancer surgery is the treatment of choice in cases where the tumor is resectable and the condition is diagnosed at an earlier stage. Bile duct tumor resection surgery is complex and should be performed by an experienced surgeon. Some of the surgical options available for bile duct cancer treatment include:
This procedure is performed to treat intrahepatic bile duct cancer. During this procedure, the doctor removes the bile duct and some parts of the liver. In some cases, it involves the removal of a complete liver lobe (hepatic lobectomy).
If the cancer is diagnosed at an early stage and is limited only to the bile duct, the patient may undergo surgery to remove the bile duct.
If the patients are diagnosed with distal bile duct cancer, they may undergo Whipple’s procedure. During this procedure, the doctor may remove the affected section of the bile duct along with some parts of the pancreas and small intestine.
It is important to remove the obstruction of the bile duct for a smooth flow of bile into the small intestine. It helps manage symptoms caused by elevated levels of bilirubin. Bile duct obstruction can be managed by placing a stent with the assistance of an endoscope.
If there is a complete blockage of the bile duct and the bile accumulates in the gallbladder, the surgeon may create a route that bypasses the blocked section of the biliary duct and delivers the bile into the intestine.
It is a technique for placing the stent to keep the biliary duct open. This procedure involves using contrast dye and X-ray imaging to detect the blocked section of the bile duct.
Radiation therapy involves guiding high-energy radiation beams onto the tumor cells to kill them. Radiation therapy, apart from treatment, may also be used as a part of palliative therapy. Following are the radiation therapies for bile duct cancer treatment:
Internal radiation therapy involves placing radiation sources, such as seeds, needles, or wires, inside or close to the tumor to kill cancer cells.
During external radiation therapy, high-energy radiation is delivered from a radiation source (a linear accelerator) placed outside the body.
During this technique, body tissue is exposed to high temperatures to make the cancer cells more sensitive to radiation therapy.
These drugs make the cancer cells more sensitive to radiation therapy and increase the efficacy of the therapy.
It involves the use of various cytotoxic and cytostatic drugs to manage bile duct cancer. It may be used along with surgery or radiation therapy as a part of bile duct cancer treatment.
Systemic chemotherapy involves the administration of the drug orally or through injection. Systemic therapy is used to kill cancer cells throughout the body.
This treatment strategy comprises the delivery of the drugs directly into the affected bile duct.
A liver transplant is the treatment in cases where the cancer is diagnosed in advanced stages and is not resectable. During this procedure, a healthy donor's liver replaces the diseased liver.
Immunotherapy is a form of systemic therapy wherein the patient’s immune system is re-engineered to identify and attack cancer cells. Immune checkpoint inhibitors, namely durvalumab and pembrolizumab, are administered as part of immunotherapy for bile duct cancer. These drugs work by binding to the immune checkpoints, which are protein molecules that block the immune system from attacking cancer cells. This reactivates the immune system to launch an attack against cancer cells.
Targeted therapy for bile duct cancer works by targeting specific cellular pathways and protein molecules on the cancer cells that allow them to divide, grow, and spread. Drugs administered as a part of targeted therapy block the growth of cancer cells or kill them.
HCG offers comprehensive diagnostic and treatment facilities for patients with bile duct cancer. The center has advanced diagnostic facilities, including laboratory testing and imaging. Medical oncologists and surgeons are extensively experienced in managing complex bile duct cancer cases. The center has world-class surgery facilities to ensure improved outcomes.
Bile duct cancer is a serious condition with a poor prognosis, especially if diagnosed at advanced stages. Medical intervention for bile duct cancer, especially surgery, is complex, and this is found to negatively impact the quality of life. Thus, it is important to identify the different bile duct cancer risk factors and adopt measures to mitigate the overall risk of this cancer type.
While anyone can be diagnosed with bile duct cancer, some individuals have a higher chance of developing this cancer type. This is because they carry certain risk factors that increase the chances of developing bile duct cancer. It is important to note that having these risk factors does not guarantee a bile duct cancer diagnosis but only increases one’s risk. The following are the important bile duct cancer risk factors:
Prolonged exposure to certain hazardous chemicals can increase one’s bile duct cancer risk. Occupational cholangiocarcinoma is a type of bile duct cancer that occurs due to chronic exposure to carcinogenic chemicals during occupation. Chemicals that increase bile duct cancer risk are nitrosamines, dioxins, and polychlorinated biphenyls. People working in automotive industries and rubber plants have a higher risk of developing bile duct cancer.
Chronic liver diseases are one of the most common bile duct cancer risk factors. It is due to chronic inflammation of the liver and bile ducts, leading to genetic changes in these cells. Primary sclerosing cholangitis is a condition characterized by scar tissue in the liver. It enhances the risk of bile duct cancer. Other chronic liver diseases that increase bile duct cancer risk are non-alcoholic fatty liver disease, bile duct stones, liver cirrhosis, and hepatitis B and C infections.
Several congenital liver and bile duct conditions increase the risk of bile duct cancer. Caroli's disease is a rare inherited disorder that involves cystic dilatation of the intrahepatic duct. Biliary papillomatosis and Lynch syndrome are genetic conditions that are identified as bile duct cancer risk factors.
An inflammatory disease of the bile duct is also a bile duct cancer risk factor. Bile duct inflammation is known as cholangitis. Infection is the most common cause of cholangitis. However, the other conditions that may cause cholangitis include blood clots, tumors, and pancreatic swelling. Choledocholithiasis is also a bile duct cancer risk factor.
Parasitic liver disease is another bile duct cancer risk factor, especially in areas where liver flukes are common. The most common liver fluke species that may potentially cause bile duct cancer include Clonorchis sinensis and Opisthorchis viverrini. These parasites increase the risk of bile duct cancer through chronic irritation, inflammation, and increased cellular turnover.
Age is a non-modifiable bile duct cancer risk factor. Although bile duct cancer may occur at any age, it is relatively more common in people over the age of 60. The average age of diagnosis for intrahepatic bile duct cancer is reported to be about 70 years.
Those with a positive family history, wherein one or more of their immediate family members have bile duct cancer, have a higher chance of developing bile duct cancer than those who do not have a family history of this condition. However, the family history risk is quite low due to the rarity of the disease.
Several underlying medical conditions reportedly increase the risk of bile duct cancer. Obese people have a higher chance of developing bile duct cancer, as these patients have a high risk of gallstones, bile duct stones, and non-alcoholic fatty liver disease. Other conditions that are associated with bile duct cancer risk include inflammatory bowel disease and diabetes.
Excessive consumption of alcohol for a significant period, along with tobacco consumption, may increase the risk of bile duct cancer.
Several people, especially those with high bile duct cancer risk, ask about how to prevent bile duct cancer or how to avoid bile duct cancer. Complete bile duct cancer prevention may not be possible; however, certain strategies may help lower its risk. The following are some of the effective bile duct cancer prevention measures:
Maintaining a healthy weight is an important bile duct cancer prevention measure. People with obesity are prone to developing various bile duct and liver-related conditions. These include NAFLD, gallstones, and bile duct stones. Further, obese people are at risk of developing insulin resistance, leading to diabetes. Diabetes is another risk factor for bile duct cancer.
Limiting the consumption of alcohol can increase the chances of bile duct cancer prevention. People with underlying liver diseases should completely avoid its consumption.
Some chemicals increase the risk of bile duct cancer, especially during occupational activities. As a bile duct cancer prevention measure, one must prevent chronic exposure to these chemicals with the help of protective equipment, such as face shields, chemical-resistant gloves, chemical-resistant glasses, respiratory equipment, shoe covers, etc.
Those diagnosed with bile duct cancer should carefully plan their diet, as certain foods can aggravate their condition and cause various problems. Some of the foods to avoid with bile duct cancer are:
Those with bile duct cancer should avoid fried and fatty foods, as these foods are difficult to digest. These foods aggravate the symptoms of bile duct cancer.
Patients with bile duct cancer may experience diarrhea due to inadequate fat digestion. High-fiber foods may worsen diarrhea and, thus, should be avoided.
Caffeine may cause gastric discomfort and, therefore, should not be consumed by patients with bile duct cancer.
Certain supplements may help in managing the symptoms of bile duct cancer. Protein supplements can manage fatigue and weakness, while enzyme supplements may assist digestion. However, before consuming any supplements, it is important to check with the doctor.
Bile duct cancer is a rare condition characterized by malignant cells in the bile duct. There are several bile duct cancer types, depending on the location of the cancer. These include intrahepatic cholangiocarcinoma, extrahepatic cholangiocarcinoma, and hilar cholangiocarcinoma. Extrahepatic cholangiocarcinoma is further divided into perihilar bile duct carcinoma and distal cholangiocarcinoma. Lastly, bile duct carcinoma is staged between 0 and 4, depending on the extent of the disease’s spread, with 0 referring to the early stage and 4 referring to the advanced stage of the disease.
