Cancer originating in the bile ducts, the tubes that carry digestive fluid (bile) from the liver, needs expert approaches to diagnosis and care.
What is cholangiocarcinoma?
Cholangiocarcinoma is a type of cancer that forms in the bile ducts. Bile ducts are thin tubes that carry bile, a digestive fluid, from the liver and gallbladder to the small intestine. Cholangiocarcinoma can occur in any part of the bile ducts, and it's classified based on its location:
- Intrahepatic: Occurs in the bile ducts inside the liver.
- Perihilar (Hilar): Occurs in the bile ducts just outside the liver.
- Distal: Occurs in the bile ducts further down, closer to the small intestine.
Cholangiocarcinoma often occurs in people older than 50 but can be diagnosed at any age. The American Cancer Society notes that it is rare, with about 8,000 people in the U.S. being diagnosed with it annually. It is believed that the number of cases may be higher because cholangiocarcinoma is hard to diagnose and may be classified as another type of cancer.
AHN Liver, Bile Duct, and Gallbladder Cancer Center of Excellence
At our Liver, Bile Duct, and Gallbladder Cancer Center of Excellence, we provide comprehensive care for patients diagnosed with hepatobiliary pancreatic (HPB) cancers such as hepatocellular carcinoma (HCC) and biliary tumors, including cholangiocarcinoma and gallbladder cancer. Understanding the complexities and challenges of these liver, bile duct, and gallbladder cancers, our multidisciplinary team — including oncologists, hepatologists, surgeons, pathologists, and radiologists — works together to develop personalized treatment plans that aim to achieve the best possible outcomes for each patient.
Why choose AHN for cholangiocarcinoma care?
When facing a cholangiocarcinoma diagnosis, choosing the right care provider is critical. AHN offers comprehensive and advanced treatment, bringing together a multidisciplinary team and cutting-edge technologies. Here are the key reasons to consider AHN for your cholangiocarcinoma care:
- Expert multidisciplinary team: Coordinated care from experienced surgeons, oncologists, radiologists, and supportive care specialists.
- Advanced technology: State-of-the-art diagnostics (CT, MRI, EUS) and treatments including minimally invasive surgery, SBRT, targeted therapies and immunotherapies, and ablation.
- Personalized treatment: Tailored plans based on individual needs, cancer stage, and preferences.
- Clinical trial access: Opportunities to participate in innovative treatment research.
- Comprehensive support: Wide range of supportive services addressing physical, emotional, and practical needs.
- Patient-centered approach: Focus on informed and involved patients.
Cholangiocarcinoma symptoms and signs
Symptoms of cholangiocarcinoma (bile duct cancer) can be vague and often don't appear until the cancer is advanced. They can vary depending on the location of the cancer within the bile ducts (intrahepatic, hilar, or distal).
Common signs and symptoms can include:
- Jaundice: Yellowing of the skin and whites of the eyes due to a buildup of bilirubin.
- Dark urine: Caused by excess bilirubin being excreted in the urine.
- Pale or clay-colored stools: Also due to a lack of bilirubin reaching the intestines.
- Abdominal pain: Often in the upper right abdomen.
- Weight loss: Unexplained and unintentional weight loss.
- Itching (pruritus): Caused by a buildup of bile salts in the skin.
- Fatigue: Feeling unusually tired.
- Fever: Can occur, especially if the bile duct is blocked and infected (cholangitis).
- Nausea and vomiting: Can occur if the bile duct is blocked.
- Enlarged liver or gallbladder: May be detected during a physical exam.
- Ascites: Fluid buildup in the abdomen.
Specific symptoms based on location:
- Intrahepatic cholangiocarcinoma: Within the liver, symptoms may be more vague and can include abdominal pain, weight loss, and fatigue. Jaundice may occur later in the disease.
- Hilar cholangiocarcinoma: At the junction of the bile ducts outside the liver, jaundice is a common early symptom.
- Distal cholangiocarcinoma: In the bile duct near the pancreas, jaundice, abdominal pain, and weight loss are common.
Causes and risk factors
Cholangiocarcinoma, also known as bile duct cancer, is a complex disease, and, in many cases, the exact cause is unknown. However, several risk factors have been identified that can increase the likelihood of developing this type of cancer. Here are some of the known causes and risk factors:
- Primary sclerosing cholangitis (PSC): This chronic liver disease, which causes inflammation and scarring of the bile ducts, is one of the strongest risk factors for cholangiocarcinoma.
- Chronic liver disease: Conditions like cirrhosis (scarring of the liver) caused by hepatitis B or C infection, or alcohol abuse, can increase the risk.
- Bile duct stones: While gallstones in the gallbladder are common, stones within the bile ducts themselves (choledocholithiasis) are less common but can increase the risk of cholangiocarcinoma.
- Liver fluke infections: In certain regions of the world, parasitic infections from liver flukes (Opisthorchis viverrini and Clonorchis sinensis), acquired through eating raw or undercooked fish, are a significant cause.
- Biliary tract abnormalities: Congenital conditions like choledochal cysts (abnormal dilations of the bile ducts) can increase the risk.
- Exposure to thorotrast: This radioactive contrast agent, used in the past for medical imaging, has been linked to an increased risk of cholangiocarcinoma many years after exposure.
- Inflammatory bowel disease (IBD): People with ulcerative colitis have a slightly higher risk of developing cholangiocarcinoma.
- Diabetes: Some studies have suggested a possible link between diabetes and an increased risk of cholangiocarcinoma.
- Obesity: Obesity is associated with increased risk of several cancers, including cholangiocarcinoma.
- Genetic factors: Certain genetic mutations and inherited conditions may increase the risk, but these are relatively rare.
- Other liver diseases: Nonalcoholic fatty liver disease (NAFLD) and nonalcoholic steatohepatitis (NASH) have been associated with an increased risk.
Cholangiocarcinoma screening and diagnosis
There is no widely recommended screening program for cholangiocarcinoma in the general population. This is because the disease is relatively rare, and effective screening tests are lacking.
Screening may be considered for individuals at high risk due to specific conditions such as primary sclerosing cholangitis (PSC) or liver fluke infection.
A medical history and physical exam is the first step in screening. The doctor will ask about your medical history, risk factors, and symptoms. A physical exam may reveal signs such as jaundice or an enlarged liver.
Blood tests
Blood tests can play a role in the detection and management of cholangiocarcinoma, but they are not typically used as a stand-alone diagnostic tool. Here's how they can be helpful:
- Liver function tests (LFTs): Elevated levels of bilirubin, alkaline phosphatase (ALP), and gamma-glutamyl transferase (GGT) can indicate a bile duct obstruction.
- Tumor markers:
- CA 19-9: A carbohydrate antigen that is often elevated in cholangiocarcinoma, but it can also be elevated in other conditions.
- CEA (carcinoembryonic antigen): Another tumor marker that may be elevated, but is less specific than CA 19-9.
Imaging tests
Imaging tests are crucial in the detection, diagnosis, staging, and monitoring of cholangiocarcinoma. They provide detailed pictures of the bile ducts, liver, and surrounding structures, allowing doctors to identify abnormalities and assess the extent of the disease. Here's how different imaging tests are used:
- Ultrasound: Can detect abnormalities in the liver and bile ducts, but may not be able to visualize small tumors.
- Computed tomography (CT) scan: Provides detailed images of the liver, bile ducts, and surrounding tissues. Often used to assess the size and location of the tumor and to look for spread to lymph nodes or distant sites.
- Magnetic resonance imaging (MRI): Offers excellent soft tissue contrast and is particularly useful for visualizing the bile ducts and detecting small tumors.
- Magnetic resonance cholangiopancreatography (MRCP): A noninvasive MRI technique that provides detailed images of the bile ducts and pancreatic ducts.
- Endoscopic ultrasound (EUS): Combines ultrasound with endoscopy to provide detailed images of the bile ducts and surrounding structures. Allows for tissue biopsies to be taken.
- Cholangiography:
- Endoscopic retrograde cholangiopancreatography (ERCP): A procedure where a flexible tube with a camera is inserted through the mouth into the bile ducts. It allows for visualization of the bile ducts, tissue biopsies, and placement of stents to relieve blockages.
- Percutaneous transhepatic cholangiography (PTC): A procedure where a needle is inserted through the skin into the bile ducts to inject contrast dye and X-rays are taken. It can be used to visualize the bile ducts, take tissue samples, and place stents.
Biopsy
A biopsy is the only way to confirm the diagnosis of cholangiocarcinoma. Tissue samples can be obtained through:
- ERCP with brushings or biopsies: Cells are collected from the bile ducts using a brush or small forceps during ERCP.
- EUS-guided fine needle aspiration (FNA): A needle is inserted through the wall of the digestive tract to collect tissue samples from the tumor.
- Percutaneous liver biopsy: A needle is inserted through the skin into the liver to collect tissue samples.
Types and stages of cholangiocarcinoma
Cholangiocarcinoma, or bile duct cancer, is classified based on its location within the bile ducts.
Intrahepatic cholangiocarcinoma occurs within the bile ducts inside the liver. It represents a smaller portion of cholangiocarcinoma cases and can be difficult to detect early, as symptoms may be vague.
Extrahepatic cholangiocarcinoma occurs in the bile ducts outside the liver. It can be further divided into hilar and distal:
- Hilar cholangiocarcinoma (Klatskin tumor): The most common type of cholangiocarcinoma, accounting for over half of all cases. Occurs at the hilum, where the left and right hepatic ducts join to form the common bile duct.
- Distal cholangiocarcinoma: Occurs in the bile duct near the pancreas.
Staging is the process of determining how far the cancer has spread. It's a critical factor in determining the best treatment options and predicting prognosis. The most common staging system used for cholangiocarcinoma is the TNM system, developed by the American Joint Committee on Cancer (AJCC). The TNM system considers three key factors:
- T (Tumor): This describes the size and extent of the primary tumor. How far has it grown into the bile duct wall, and has it spread to nearby structures?
- N (Nodes): This indicates whether the cancer has spread to nearby lymph nodes. If so, how many lymph nodes are affected?
- M (Metastasis): This indicates whether the cancer has spread to distant sites or organs, such as the liver, lungs, or bones.
Once the T, N, and M categories are determined, they are combined to assign an overall stage. The stage groupings range from Stage 0 (the earliest stage) to Stage IV (the most advanced stage). Here's a very simplified overview:
- Stage zero: Carcinoma in situ (very early stage).
- Stage one: Early-stage tumor, confined to the bile duct.
- Stage two: Tumor has grown further, possibly into nearby structures.
- Stage three: Tumor has spread to regional lymph nodes.
- Stage four A: Tumor has spread to adjacent structures.
- Stage four B: The tumor has spread to more distant parts of the body.
Generally, earlier stages have a better prognosis (outlook) than later stages. However, prognosis also depends on other factors, such as your overall health, the tumor's makeup, and the treatment received. Your AHN care team is here to walk you through the specifics of your diagnosis and help you feel confident in your treatment.
Cholangiocarcinoma treatment
Treatment for cholangiocarcinoma depends on several factors, including the location and stage of the cancer, the patient's overall health, and individual preferences. The primary goal is to remove or control the cancer, relieve symptoms, and improve the patient's quality of life.
Surgery
Surgery is a primary treatment option for cholangiocarcinoma, and, in many cases, it offers the best chance for long-term survival. The goal of surgery is to remove the tumor completely, along with a margin of healthy tissue, to ensure that all cancer cells are eliminated. Here's how surgery helps in the treatment of cholangiocarcinoma:
- Resection: If the cancer is localized and hasn't spread, surgical removal (resection) of the tumor and surrounding tissue is the most effective treatment. The type of surgery depends on the location of the tumor:
- Partial hepatectomy: Removal of a portion of the liver for intrahepatic cholangiocarcinoma.
- Whipple procedure (pancreaticoduodenectomy): For distal cholangiocarcinoma, involving removal of the head of the pancreas, duodenum, gallbladder, and bile duct. Learn more about Whipple procedures.
- Bile duct resection: Removal of the affected portion of the bile duct for hilar or extrahepatic cholangiocarcinoma, often with reconstruction of the bile duct.
- Liver transplant: In select cases of early-stage intrahepatic cholangiocarcinoma, liver transplantation may be an option. However, it's not typically used for extrahepatic cholangiocarcinoma due to the higher risk of recurrence.
Radiation therapy
Radiation therapy uses high-energy beams, such as X-rays or protons, to kill cancer cells. While surgery is often the primary treatment for cholangiocarcinoma when possible, radiation therapy plays a significant role in specific situations. Radiation therapy may include:
- External beam radiation therapy (EBRT): Delivers radiation from outside the body to the tumor.
- Brachytherapy (internal radiation therapy): Involves placing radioactive material directly into or near the tumor.
- Stereotactic body radiation therapy (SBRT): Delivers high doses of radiation to a small area, minimizing damage to surrounding tissues.
- Palliative radiation therapy: Used to relieve symptoms such as pain or blockage.
Chemotherapy
Chemotherapy plays a significant role in treating cholangiocarcinoma, although it's often used in combination with other treatments like surgery and radiation therapy. Chemotherapy involves using drugs to kill cancer cells throughout the body. Here's how it helps in the treatment of cholangiocarcinoma:
- Adjuvant chemotherapy: Given after surgery to kill any remaining cancer cells and reduce the risk of recurrence.
- Neoadjuvant chemotherapy: Given before surgery to shrink the tumor, making it easier to remove.
- Palliative chemotherapy: Used to slow the growth of advanced cancer, relieve symptoms, and improve quality of life when surgery is not possible.
- Common chemotherapy drugs: Gemcitabine, cisplatin, fluorouracil (5-FU), oxaliplatin.
Targeted Therapy
Targeted therapies are drugs that specifically target certain molecules or pathways involved in cancer growth. FGFR inhibitors (e.g., pemigatinib, infigratinib) may be used for cholangiocarcinomas with FGFR2 gene fusions or rearrangements. IDH1 inhibitors (e.g., ivosidenib) may be used for cholangiocarcinomas with IDH1 mutations.
Immunotherapy
Immune checkpoint inhibitors help the immune system recognize and attack cancer cells. Pembrolizumab may be used in some cases of advanced cholangiocarcinoma, particularly if the cancer has certain genetic characteristics (e.g., high microsatellite instability or mismatch repair deficiency).
Biliary drainage
Biliary drainage is a procedure performed to relieve a blockage in the bile ducts. In the context of cholangiocarcinoma, which often causes obstruction of the bile ducts, biliary drainage can provide significant benefits. Here's how biliary drainage can help treat cholangiocarcinoma:
- Stenting: If the bile duct is blocked, a stent (a small tube) can be placed to keep it open and allow bile to drain.
- Percutaneous biliary drainage (PBD): A catheter is inserted through the skin into the bile duct to drain bile.
Ablation
Ablation is a technique used to destroy tumors, including cholangiocarcinoma, using heat, cold, or other energy forms. It's generally considered for patients who are not candidates for surgery or as a complementary treatment alongside surgery.
- Radiofrequency ablation (RFA): Uses heat to destroy cancer cells.
- Microwave ablation: Similar to RFA, but uses microwaves to generate heat.
- Irreversible electroporation (IRE): Uses electrical pulses to create pores in cancer cells, leading to cell death.
Cholangiocarcinoma FAQs
A cholangiocarcinoma diagnosis can bring about questions and, possibly, concerns. Your AHN care team is here to help and will be your best resource to provide specific answers to your questions and help you feel confident in your treatment options. To help you get some background information that may aid your discussions, we’ve provided answers to some frequently asked questions many patients have.
What is the survival rate for cholangiocarcinoma?
The survival rate for cholangiocarcinoma varies significantly depending on several factors, including the stage of the cancer at diagnosis, the location of the tumor (intrahepatic, perihilar, or distal), the patient's overall health, and the treatment received. It's important to remember that survival rates are statistical averages and cannot predict the outcome for any individual. Here's a general overview based on the five-year relative survival rates from the National Cancer Institute's SEER database:
- Localized: If the cancer is found early and is localized (confined to the bile ducts or liver), the five-year survival rate is around 25 – 30%. Surgical resection offers the best chance of long-term survival in these cases.
- Regional: If the cancer has spread to nearby lymph nodes or tissues, the five-year survival rate is approximately 10 – 15%.
- Distant: If the cancer has metastasized (spread to distant organs), the five-year survival rate is very low, generally around 2%.
It's also important to note that these numbers are based on data collected over several years and include all patients regardless of their treatment. Advances in treatment may improve survival rates over time. Survival rates are often higher in people who are eligible for surgical resection. Those who undergo curative resection have higher rates of survival.
Is bile duct cancer aggressive?
Yes, cholangiocarcinoma is generally considered an aggressive cancer. Here's why:
- Late diagnosis: It is often diagnosed at a late stage because early symptoms can be vague or non-specific, leading to delays in diagnosis and treatment. By the time it's detected, it may have already spread.
- Difficult to treat: Cholangiocarcinoma can be challenging to treat effectively, especially when it is advanced. Complete surgical removal is often the best chance for long-term survival, but it is not always possible due to the tumor's location or extent.
- Rapid progression: The cancer can grow and spread relatively quickly, making it difficult to control.
- Resistance to therapy: Cholangiocarcinoma can develop resistance to chemotherapy and radiation therapy, limiting the effectiveness of these treatments.
- High recurrence rate: Even after successful surgical removal, there's a significant risk of the cancer recurring.
What causes death in cholangiocarcinoma patients?
The cause of death in cholangiocarcinoma patients is usually related to complications from the cancer itself or the progression of the disease. Common causes include:
- Liver failure: As the cancer grows and obstructs the bile ducts, it can lead to liver damage and eventually liver failure.
- Sepsis/infection: Bile duct obstruction can lead to cholangitis (infection of the bile ducts), which can spread to the bloodstream and cause sepsis, a life-threatening condition.
- Malnutrition and cachexia: Cancer can cause loss of appetite, weight loss, and muscle wasting (cachexia), which can weaken the body and make it more susceptible to complications.
- Metastatic disease: The spread of cancer to distant organs (e.g., liver, lungs, bones) can cause organ failure and other complications that lead to death.
- Complications from treatment: While treatments like surgery, chemotherapy, and radiation therapy can help control the cancer, they can also cause side effects that contribute to death in some cases.
- Bile duct obstruction: The blockage of bile ducts can lead to serious complications.
It's important to remember that individual experiences can vary, and advancements in treatment are continuously being made to improve outcomes for cholangiocarcinoma patients.
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Second opinions
If you have cancer, you have a team of oncology specialists ready to review your medical records and offer you a second opinion. After completing their review, they’ll talk with you about your goals to determine a course of treatment that’s right for you. To get started, fill out our Second Opinion Request form. A nurse navigator will contact you within the next 24 to 48 hours to discuss next steps and schedule.