What is hilar cholangiocarcinoma?
Hilar cholangiocarcinoma is a type of bile duct cancer that occurs in the bile ducts that lead out of the liver (hepatic ducts) and join with the gallbladder. Hilar cholangiocarcinomas are also known as Klatskin tumors.
What is cholangiocarcinoma (bile duct cancer)?
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%. Cholangiocarcinoma (bile duct cancer) is a rare cancer that occurs when cells in a bile duct grow abnormally and out of control. What Are Symptoms of Cholangiocarcinoma?
What is perihilar bile duct cancer?
Perihilar (also called hilar) bile duct cancers. These cancers start at the hilum, where the left and right hepatic ducts have joined and are just leaving the liver. These are also called Klatskin tumors. These cancers are grouped with distal bile duct cancers as extrahepatic bile duct cancers.
What are the different types of cholangiocarcinoma?
Doctors divide cholangiocarcinoma into different types based on where the cancer occurs in the bile ducts: Intrahepatic cholangiocarcinoma occurs in the parts of the bile ducts within the liver and is sometimes classified as a type of liver cancer. Hilar cholangiocarcinoma occurs in the bile ducts just outside of the liver.
Is hilar cholangiocarcinoma curable?
Background. Hilar cholangiocarcinoma is the most common malignant tumor affecting the extrahepatic bile duct. Surgical treatment offers the only possibility of cure, and it requires removal of all tumoral tissues with adequate resection margins.
Is hilar cholangiocarcinoma cancerous?
Hilar cholangiocarcinoma is a type of bile duct cancer that occurs in the bile ducts that lead out of the liver (hepatic ducts) and join with the gallbladder. Hilar cholangiocarcinomas are also known as Klatskin tumors.
What causes hilar cholangiocarcinoma?
Cholangiocarcinoma happens when cells in the bile ducts develop changes in their DNA. A cell's DNA contains the instructions that tell a cell what to do. The changes tell the cells to multiply out of control and form a mass of cells (tumor) that can invade and destroy healthy body tissue.
How long do cholangiocarcinoma patients live?
Cholangiocarcinomas arise from the epithelial cells of intrahepatic and extrahepatic bile ducts. They generally have a very poor prognosis. Many studies report a dismal median survival of approximately 6 months.
Can you survive cholangiocarcinoma?
If the cancer is diagnosed at an early stage, the 5-year survival rate is 25%. If the cancer has spread to the regional lymph nodes, the 5-year survival rate is 8%. If the cancer has spread to a distant part of the body, the 5-year survival rate is 2%.
How rare is hilar cholangiocarcinoma?
Incidence and epidemiology Cholangiocarcinoma accounts for less than 2% of all human malignancies but is the second most common primary liver tumor (4,5). Although rare in Western countries, it is more commonly seen in Asia with incidences as high as 113 per 100,000 men and 50 per 100,000 women (6).
How do you get cholangiocarcinoma?
Several non-genetic risk factors for cholangiocarcinoma have been identified. These include a bile duct disease called primary sclerosing cholangitis, bile duct stones or cysts, and exposure to certain chemical toxins used in manufacturing.
Who is at risk for cholangiocarcinoma?
Older people are more likely than younger people to get bile duct cancer. Most people diagnosed with bile duct cancer are in their 60s or 70s.
How rare is hilar cholangiocarcinoma?
Incidence and epidemiology Cholangiocarcinoma accounts for less than 2% of all human malignancies but is the second most common primary liver tumor (4,5). Although rare in Western countries, it is more commonly seen in Asia with incidences as high as 113 per 100,000 men and 50 per 100,000 women (6).
Are Klatskin tumors cancerous?
Cancer that forms in the area where the left and right hepatic ducts join just outside the liver and form the common hepatic duct. Bile ducts carry bile from the liver and gallbladder to the small intestine. Klatskin tumor is a type of extrahepatic bile duct cancer.
What constitutes long term survival after surgery for hilar cholangiocarcinoma?
The clinicopathologic features of 32 5-year survivors were compared with patients whose survival was less than 5 years. Results: Thirty-two patients survived longer than 5 years after surgical treatment of hilar cholangiocarcinoma; 22 of these patients (69%) were still alive at last follow-up.
What are the types of cholangiocarcinoma?
The Liver Cancer Study Group of Japan (LCSGJ) used retrospective data from 245 patients to distinguish three macroscopic growth types for intrahepatic cholangiocarcinoma: mass-forming type; periductal-infiltrating type; and intraductal-growth type.
How to treat hilar cholangiocarcinoma?
Treatment typically involves surgery, liver transplant or supportive treatments to reduce your pain and other symptoms.
What is the treatment for liver cancer?
Chemotherapy and radiation are typically used before the liver transplant in order to kill as many cancer cells as possible before surgery. This treatment typically involves: Intravenous chemotherapy with a medicine that makes your cells more vulnerable to radiation. External beam radiation therapy.
What is Cholangiocarcinoma?
Cholangiocarcinoma (CCA), or bile duct cancer, is a type of rare cancer that grows in the small tubes that carry bile, a fluid that helps with digestion. These tubes are called the bile ducts. The bile ducts connect the liver to the gall bladder and small intestine.
What is the prognosis for someone with cholangiocarcinoma?
The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as:
How many types of cholangiocarcinoma are there?
There are three types of cholangiocarcinoma that are named for where they grow in the bile ducts:
How long are people alive with cholangiocarcinoma?
The survival rates also don’t take into account newer treatments. For people with extrahepatic cholangiocarcinoma, 10% are alive 5 years after diagnosis. For people with intrahepatic cholangiocarcinoma that has been found at an early stage, 24% are alive 5 years after diagnosis.
How accurate are survival rates for cholangiocarcinoma?
Because there are so few people with cholangiocarcinoma, these rates may not be very accurate. The survival rates also don’t take into account newer treatments.
Can FGFR2 be used for cholangiocarcinoma?
In these cases, a FGFR2 inhibitor (pemigatinib) can help treat cholangiocarcinoma. Immunotherapy: Immunotherapy helps the body’s immune system fight cancer cells. Doctors are studying if immunotherapy can help treat cholangiocarcinoma in clinical trials.
Can cholangiocarcinoma be diagnosed with bilirubin?
Later, when the tumor gets larger, symptoms can include: Lab Tests: If you have symptoms of cholangiocarcinoma, your doctor will order lab tests of your blood to check your bilirubin levels .
What Is Cholangiocarcinoma?
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%.
How Is Cholangiocarcinoma Diagnosed?
In addition to a patient history and physical exam, tests used to diagnose cholangiocarcinoma (bile duct cancer) or to rule out other causes for symptoms include:
What Is the Life Expectancy for Cholangiocarcinoma?
Life expectancy for cholangiocarcinoma (bile duct cancer) is often expressed in five-year survival rates, that is, how many people will be alive five years after diagnosis.
How long does bile duct cancer last?
Extrahepatic bile duct cancers (those starting outside the liver) (includes both perihilar and distal bile duct cancers) five-year survival rates: The 5-year survival for these tumors at the regional stage is slightly better than for the localized stage, although the reason for this is not clear.
What are the abnormalities of the bile duct and pancreatic duct?
Abnormalities where the bile duct and pancreatic duct normally meet. Cirrhosis of the liver. Infection with hepatitis B virus or hepatitis C virus. Polycystic liver disease. Caroli syndrome. Inflammatory bowel disease. Ulcerative colitis. Crohn’s disease. Older age: most people diagnosed with cholangiocarcinoma are in their 60s or 70s.
What is non-alcoholic fatty liver disease?
Exposure to thorium dioxide (Thorotrast), a radioactive contrast agent used for X-rays until the 1950s (it is no longer used) Family history of bile duct cancer. Diabetes. Alcohol consumption, particularly in people who have liver problems from drinking alcohol.
How much cancer spreads outside the bile ducts?
Localized (no sign that the cancer has spread outside of the bile ducts): 24%. Regional (cancer has spread outside the bile ducts to nearby structures or lymph nodes): 7%. Distant (cancer has spread to distant parts of the body such as the lungs ): 2%.
Where does cholangiocarcinoma start?
Cholangiocarcinomas start in the gland cells that line the inside of the ducts. Other types of bile duct cancers are much less common. These include sarcomas, lymphomas, and small cell cancers. Our information does not cover these rare types of bile duct cancer.
What is the name of the cancer that starts at the hilum?
Perihilar (also called hilar) bile duct cancers. These cancers start at the hilum, where the left and right hepatic ducts have joined and are just leaving the liver. These are also called Klatskin tumors. These cancers are grouped with distal bile duct cancers as extrahepatic bile duct cancers.
What are the different types of bile duct cancer?
Based on where the cancers start (see the picture below), they're grouped into 3 types: Intrahepatic bile duct cancers. Perihilar (also called hilar) bile duct cancers. Distal bile duct cancers.
Where does bile duct cancer start?
Intrahepatic bile duct cancers. These cancers start in the smaller bile duct branches inside the liver. Sometimes they're confused with cancers that start in the liver cells ( hepatocellular carcinomas ), and they are often treated the same way.
Can bile duct cancer be divided into different types?
Bile duct cancers can also be divided into types based on how the cancer cells look under the microscope.
Is liver cancer metastatic?
This type of cancer starts in the main cells that make up the liver. Cancers that start in other organs, such as the colon or rectum, can sometimes spread (metastasize) to the liver. These metastatic cancers are not true liver cancers. For example, colorectal cancer that has spread to the liver is still colorectal cancer, not liver cancer.
Is a bile duct tumor a cancer?
Not all bile duct tumors are cancer. For example, bile duct hamartomas and bile duct adenomas are types of benign (non-cancer) tumors.
Where is cholangiocarcinoma located?
Distal cholangiocarcinoma (bile duct cancer): These cancers are further down the bile duct near the pancreas and small intestine. Along with the bile duct and nearby lymph nodes, in most cases the surgeon must remove part of the pancreas and small intestine.
What is the procedure for cholangiocarcinoma?
Surgery for cholangiocarcinoma is a complex operation that depend on tumor location and extension (if the tumor has grown through the layers of the tissue in which it started ) and should be done by an experienced surgeon working at a major medical center whenever possible.
What is the name of the cancer that begins where the bile ducts leave the liver?
Perihilar cholangiocarcinoma (bile duct cancer): These cancers begin where the branches of the bile duct first leave the liver. Surgery for these cancers requires great skill, as the operation is quite extensive. Usually part of the liver is removed, along with the bile duct, gallbladder, nearby lymph nodes, and sometimes part of the pancreas and small intestine. Then the surgeon connects the remaining ducts to the small intestine. This is not an easy operation for the patient, and there can be surgical complications.
What is the best treatment for cholangiocarcinoma?
There are various treatment options for cholangiocarcinoma including surgery, radiation therapy, locoregional therapies, chemotherapy, targeted therapy, immunotherapy, or palliative therapy. The most sought after treatment for cholangiocarcinoma is surgery. Radiation therapy and chemotherapy may be used if the cancer cannot be removed with surgery ...
What kind of doctor treats cholangiocarcinoma?
In many cases, a team of doctors including a surgical oncologist, medical oncologist, radiation oncologist, interventional radiologist, gastroenterologist and hepatologist will work with the patient to determine the best treatment plan. There are various treatment options for cholangiocarcinoma including surgery, radiation therapy, ...
What happens after cholangiocarcinoma is staged?
After cholangiocarcinoma is found and staged, your cancer care team will discuss your treatment options with you. It is important for you to take time and think about your choices. The treatment of cholangiocarcinoma depends on the size and location of the tumor, whether your cancer has spread, your overall health, anticipated side effects, and the chance of curing the disease, extending life or relieving symptoms.
What is the procedure to remove a resectable liver cancer?
To treat these cancers, the surgeon cuts out the part of the liver containing the cancer. Removing part of the liver is called a partial hepatectomy.
