Bile duct cancer symptoms: Stages, outlook, and more (2023)

Bile duct cancer, also known as cholangiocarcinoma, is a rare form of cancer that affects the bile ducts. Two bile ducts in the human body connect the liver and gallbladder to the small intestine. They are important for digestion.

Bile duct cancer can start either inside the liver — intrahepatic — or outside of the liver — extrahepatic. Though very similar, the two types of bile duct cancer have somewhat different survival outlooks and may present with slightly different symptoms.

Experts break bile duct cancer down into several different stages. This helps doctors explain a person’s outlook, as well as help guide their treatment options.

This article reviews the main symptoms of bile duct cancer, early warning signs, its symptoms by stage, and more.

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According to the American Cancer Society (ACS), early stages of bile duct cancer do not tend to present with symptoms. When it does, it is usually due to a tumor blocking a bile duct.

The most common symptom of bile duct cancer is jaundice, which is a yellowing of the eyes and skin.

However, bile duct cancer can cause other symptoms, such as:

  • itchy skin
  • abdominal pain
  • a fever
  • vomiting
  • nausea
  • weight loss
  • loss of appetite
  • greasy, pale stools
  • dark urine

Bile duct cancer is difficult to catch early. This is partly because the bile ducts are hard to reach, buried deep within the abdomen. Doctors cannot feel for a lump or mass during a routine exam, nor can they see one on imaging scans.

There are also no reliable blood tests to check for bile duct cancer.

Finally, bile duct cancer typically does not show early warning signs or symptoms unless a tumor has blocked a bile duct.

Bile duct cancer is a rare form of cancer, which means that many of the associated symptoms are more likely the result of another condition affecting the liver.

(Video) Bile Duct Cancer: A patient's journey

Doctors typically describe cancer in terms of its stage.

Staging provides doctors with a way to easily describe the cancer’s progression, as well as by helping them determine the best available treatment options.

Bile duct cancer can be intrahepatic or extrahepatic. Extrahepatic includes perihilar and distal bile duct cancers.

(Video) Bile duct cancer: Mayo Clinic Radio

The table below describes the stages for each type of bile duct cancer, and what symptoms may present at each stage, according to the National Cancer Institute (NCI) and ACS.

StagesIntrahepatic bile duct cancerPerihilar bile duct cancerDistal bile duct cancer Symptoms
1 Cancer has formed inside the tissue lining the intrahepatic bile duct. Substages are:
1A: The tumor is 5 centimeters (cm) or smaller.
1B: The tumor is larger than 5 cm.
Cancer has formed in the innermost layer of tissue lining the perihilar bile duct.
It has spread into the muscle layer or fibrous tissue layer of the perihilar bile duct wall.
Cancer has formed in the innermost layer of the lining of the bile duct.
It has spread fewer than 5 millimeters (mm) into the wall of the distal bile duct.
Symptoms are not likely to occur in these early stages.
2Either:
The tumor has spread through the wall of an intrahepatic bile duct and into a nearby blood vessel.
Or:
More than one tumor has formed in the intrahepatic bile duct and may have spread into a blood vessel.
Cancer has spread through the wall of the perihilar bile duct to nearby fatty tissue or liver tissue.Stage 2 has two substages:
2A: Cancer has spread fewer than 5 mm into the wall of the distal bile duct and has spread to 1–3 nearby lymph nodes or 5–12 mm into the wall of the distal bile duct
2B: Cancer has spread 5 mm or more into the wall of the distal bile duct. Cancer may have spread to 1–3 nearby lymph nodes.
Symptoms are not likely to occur in these early stages.
3Stage 3 has two substages:
3A: Tumor has spread through the outer lining of the liver.
3B: Cancer has spread to organs or tissues near the liver, such as the:
• colon
• stomach
• duodenum
• diaphragm
• common bile duct
• abdominal wall
Or,
The cancer has spread to nearby lymph nodes.
Stage 3 has three substages:
3A: Cancer has spread to branches on one side of the hepatic artery or of the portal vein.
3B: Cancer has spread to other areas, such as the:
• common hepatic artery
• portal vein
• hepatic ducts
3C: Cancer has spread to 1–3 nearby lymph nodes.
Stage 3 has two substages:
3A: Cancer has spread into the wall of the distal bile duct and to 4 or more nearby lymph nodes.
3B: Cancer has spread to the large vessels that carry blood to the organs in the abdomen. It may also have spread to 1 or more nearby lymph nodes.
Symptoms may present at this stage as the tumor has grown in size and may block the bile duct. Symptoms may include:
• jaundice
• dark urine
• itchy skin
• abdominal pain
• nausea and vomiting
4Cancer has spread to most of the organs in the abdomen and distant parts of the body, such as:
• bones
• lungs
• distant lymph nodes
Stage 4 has two substages:
4A: Cancer has spread to 4 or more nearby lymph nodes.
4B: Cancer has spread to most of the organs in the abdomen and other parts of the body, which could include the:
• liver
• distant lymph nodes
• bones
• brain
• lungs
• skin
Cancer has spread to most of the organs in the abdomen and other parts of the body.
This may include the liver or lungs.
A person may experience both symptoms of bile duct cancer and other symptoms related to the area in which it has spread.

A person should talk with a doctor if they notice jaundice, yellowing of the eyes and skin. This can indicate that something is wrong with their liver.

However, jaundice can occur due to several different issues that affect the liver. As bile duct cancer is rare, jaundice is more likely to result from a liver problem such as hepatitis, cirrhosis, or alcohol-related liver disease.

It is difficult to detect bile duct cancer before it has spread enough to cause symptoms. There are no reliable screening tests to detect bile duct cancer early.

If a person has symptoms of a liver problem and a doctor rules out other, more common causes, they may search for the presence of bile duct cancer.

(Video) Newly Diagnosed: Bile Duct Cancer

To diagnose bile duct cancer, a doctor will review both family and personal medical history. If they suspect cancer, they may order a number of tests, which may include one or more of the following:

  • angiography, which is a type of x-ray
  • cholangiography, a test that looks for blocks in the bile duct
  • MRI scan
  • CT scan
  • biopsy
  • blood tests to look for tumor markers and check liver functioning
  • ultrasounds

Once doctors find bile duct cancer, it has usually progressed too far for surgical intervention. This makes the overall prognosis for bile duct cancer low.

Doctors often use 5-year survival rates to help give an idea of the person’s overall outlook. Doctors base these estimates on data available about the number of people still alive following a diagnosis of different stages of bile duct cancer.

While they can be helpful, it is important to note that they do not provide the full picture. A person’s overall health, age, and other factors can increase or decrease a person’s chance of survival.

For intrahepatic bile duct cancers, the following 5-year survival rates based on stage are:

  • Localized: 24%
  • Regional, or spread to nearby tissue or lymph nodes: 9%
  • Distant, or spread to far away organs: 2%
  • All stages combined: 9%

For extrahepatic bile duct cancers, the following 5-year survival rates based on stage are:

  • Localized: 17%
  • Regional, or spread to nearby tissue or lymph nodes: 16%
  • Distant, or spread to far away organs: 2%
  • All stages combined: 10%

Frequently asked questions

(Video) Interview with Bile Duct Cancer Survivor Lisa Craine

The following sections provide answers to frequently asked questions about bile duct cancer.

Can you have bile duct cancer with no symptoms?

Yes, the early stages of bile duct cancer have no obvious signs or symptoms unless a tumor blocks a duct. Bile duct cancer may only start to display obvious symptoms in stages 3 or 4.

The most common sign of bile duct cancer is jaundice, but this can also be the result of several different conditions that affect the liver.

Would bile duct cancer show up in blood tests?

One of the issues with finding bile duct cancer early is that no reliable blood or visual tests can check for its presence.

During diagnosis, blood tests may help doctors determine the livers’ function and help them find tumor markers, but doctors will need to perform additional tests to detect bile duct cancer.

How do you know if bile duct cancer has spread?

A doctor will need to test for the presence of cancer in other areas of the body.

If the cancer has spread, a person may experience other signs and symptoms related to the location to which it has spread.

Bile duct cancer starts in the bile duct and may appear inside or outside the liver.

Bile duct cancer does not typically show signs or symptoms until it blocks a bile duct in its later stages. A person is unlikely to discover a tumor until they show symptoms of bile duct cancer and go in for an examination.

(Video) Understanding Liver and Bile Duct Cancer

Diagnosis typically involves the use of several tests, including MRI and CT scans.

FAQs

How long do you live with Stage 4 bile duct cancer? ›

For distant intrahepatic bile duct cancers, which have spread to distant areas or organs (stage 4B), the five-year relative survival rate is 2 percent.

What is the life expectancy for someone with bile duct cancer? ›

If the cancer is diagnosed at an early stage, the 5-year relative survival rate is 24%. If the cancer has spread to the regional lymph nodes, the 5-year relative survival rate is 9%. If the cancer has spread to a distant part of the body, the 5-year relative survival rate is 2%.

What are the symptoms of cholangiocarcinoma stage 4? ›

For advanced bile duct cancer, symptoms might include:
  • yellowing of skin and whites of eyes and itchy skin (jaundice)
  • abdominal (tummy) pain.
  • feeling or being sick.
  • unexplained weight loss.
  • mood changes.

How does bile duct cancer cause death? ›

Untreated, death occurs within a few months of presentation, mostly attributable to biliary sepsis, liver failure or hemorrhage. These causes of death are mostly sequelae of local tumor effects, and metastatic spread is rare at the time of presentation.

Is there any hope for bile duct cancer? ›

Whenever possible, surgery is the main treatment for bile duct cancers. It offers the only realistic chance for a cure.

How does bile duct cancer progress? ›

If a tumor grows through the bile duct wall, it can invade (grow into) nearby blood vessels, organs, and other structures. It might also grow into nearby lymphatic or blood vessels, and from there spread to nearby lymph nodes or to other parts of the body.

Is cholangiocarcinoma fast growing? ›

Cholangiocarcinomas are usually slow-growing tumors that spread locally via the lymphatic system. Treatment and long-term prognosis are dependent upon the location of the mass.

How painful is bile duct cancer? ›

Abdominal (belly) pain

Early bile duct cancers seldom cause pain, but bigger tumors may cause belly pain, especially below the ribs on the right side.

How aggressive is bile duct cancer? ›

Cholangiocarcinoma is extremely aggressive. Fewer than 10% of patients survive five years after diagnosis. Survival is even lower for patients diagnosed at a metastatic stage.

How many stages are there in bile duct cancer? ›

The number staging system divides bile duct cancers into 4 main stages, from 1 to 4. The staging is different depending on the type of bile duct cancer you have.

Do you lose your appetite with bile duct cancer? ›

Symptoms of bile duct cancer can include: your skin or the whites of your eyes turn yellow (jaundice), you may also have itchy skin, darker pee and paler poo than usual. loss of appetite or losing weight without trying to. feeling generally unwell.

Is cholangiocarcinoma always fatal? ›

Cholangiocarcinoma (bile duct cancer) is a deadly disease. Even when it's detected early, the five year survival rates for people with this cancer is less than 25%.

Can you go into remission with cholangiocarcinoma? ›

Cholangiocarcinoma recurrence is possible after a patient completes an initial course of treatment and is found to be cancer-free (in remission) for a period of time.

What is the most common presentation of cholangiocarcinoma? ›

Patients will present with jaundice, along with associated pruritus, steatorrhoea, non-specific abdominal pain, or dark urine. In advanced disease, weight loss and lethargy can occur. On examination, jaundice and cachexia are often evident.

What organs does bile duct cancer spread to? ›

Advanced bile duct cancer is cancer that has spread outside the bile ducts into lymph nodes or organs near the bile duct.
...
The most common places for bile duct cancer to spread are the:
  • lungs.
  • bones.
  • lining of the abdomen (peritoneum)
  • brain.
Nov 15, 2021

What organs does bile duct cancer affect? ›

Bile duct cancer is a rare disease in which malignant (cancer) cells form in the bile ducts. Bile duct cancer is also called cholangiocarcinoma. A network of tubes, called ducts, connects the liver, gallbladder, and small intestine.

Where does bile duct cancer hurt? ›

Pain in the abdomen is a common symptom of bile duct cancer. The pain is usually on your right hand side, just below your ribs. It's important to remember that this type of pain can be caused by other less serious conditions, such as gallstones.

What is the latest treatment for bile duct cancer? ›

Ivosidenib is an oral medication that is given once a day with or without food. In 2022, the FDA approved dabrafenib (Tafinlar) in combination with trametinib (Mekinist) for the treatment of people with biliary tract cancer with a BRAF V600E mutation that is either metastatic or that cannot be removed with surgery.

Does chemo work for bile duct cancer? ›

Systemic chemotherapy is used to treat unresectable, metastatic, or recurrent bile duct cancer. The following chemotherapy drugs may be used: gemcitabine and cisplatin. capecitabine and oxaliplatin (XELOX)

What is the best hospital for bile duct cancer? ›

Mayo Clinic is one of the major referral centers in the United States for primary sclerosing cholangitis, which is the major risk factor for bile duct cancer. Mayo Clinic surgeons pioneered the use of liver transplant to treat hilar cholangiocarcinoma.

What is considered early stage bile duct cancer? ›

Early bile duct cancer may be defined as a carcinoma whose invasion is confined within the fibromuscular layer of the extrahepatic bile duct or intrahepatic large bile duct without distant metastasis irrespective of lymph node involvement.

Is there a cure for stage 4 bile duct cancer? ›

Stage 4 is the most advanced stage of bile duct cancer, meaning the disease has spread to other parts of the body. There is no cure for stage 4 bile duct cancer. In most cases, treatment focuses on reducing symptoms and improving a person's quality of life.

Can chemo help stage 4 bile duct cancer? ›

Chemotherapy is a common treatment for people with advanced bile duct cancer. This means that the cancer has spread outside the bile ducts or has come back sometime after you were first treated.

Is bile duct cancer very painful? ›

Abdominal (belly) pain

Early bile duct cancers seldom cause pain, but bigger tumors may cause belly pain, especially below the ribs on the right side.

How long can you live with Stage 4 C? ›

Although the overall prognosis may be poor based on cases with previous patients and older treatments, many patients with stage 4 cancer can live for years.

Can you go into remission with bile duct cancer? ›

Although bile duct cancer is curable if doctors diagnose it early, the goal for most people with the disease is remission. Healthcare professionals consider a cancer “cured” when no more cancerous cells remain in the body and they do not expect the disease to recur.

Does Chemo work for bile duct cancer? ›

Systemic chemotherapy is used to treat unresectable, metastatic, or recurrent bile duct cancer. The following chemotherapy drugs may be used: gemcitabine and cisplatin. capecitabine and oxaliplatin (XELOX)

Where does bile duct cancer spread to first? ›

The most common places for bile duct cancer to spread are the: lungs. bones. lining of the abdomen (peritoneum)

Videos

1. Medical advances improve bile duct cancer outcomes
(MD Anderson Cancer Center)
2. Bile Duct Cancer: Klatskin Tumors
(Mark Fraiman, MD - St Joseph Medical Center Liver and Pancreas Center)
3. Surviving Cholangiocarcinoma - a Patient Story
(BostonScientificEndo)
4. Newlyweds & Newly Diagnosed // Bile Duct Cancer // Cholangiocarcinoma // Join Us Along Our Journey
(Madee Lee)
5. What is the Survival Rate of Liver Cancer?
(Roswell Park Comprehensive Cancer Center)
6. Stage 4 Liver Cancer Symptoms
(disease and healthy life)
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