Sri Shankara Cancer Hospital & Research Centre

Biliary and Gallbladder Cancer: A Comprehensive Guide

This information is intended to provide a general overview of Biliary and Gallbladder Cancer. It is not a substitute for professional medical advice, diagnosis, or treatment. Always consult with a qualified healthcare provider for any health concerns or before making any decisions related to your health.

General Information

Biliary cancers (also known as cholangiocarcinoma or bile duct cancers) and gallbladder cancer are relatively uncommon. These cancers are named according to their site of origin within the biliary system.

The Biliary System

The biliary system is responsible for producing bile, a fluid essential for digestion. Bile is created in the liver and stored in the gallbladder. The bile ducts are tree-like structures that transport bile from the liver to the duodenum (the upper part of the small intestine).

Types of Biliary and Gallbladder Cancer

Biliary cancers are classified based on where they form:

Intrahepatic Bile Duct Cancer (Intrahepatic Cholangiocarcinoma)

This cancer develops in the bile ducts inside the liver. It accounts for approximately 10% of bile duct cancer cases and is sometimes mistaken for liver cancer. Although rare, the incidence of this type is increasing globally.

Extrahepatic Bile Duct Cancers

These cancers occur in the bile ducts outside the liver and include two primary types:

Perihilar Bile Duct Cancer (Klatskin Tumour or Perihilar Cholangiocarcinoma): The most common extrahepatic type, accounting for 40% to 60% of all bile duct cancers. It forms where the main bile ducts exit the liver.
Distal Bile Duct Cancer: This cancer begins in the part of the bile duct that passes through the pancreas and connects to the small intestine, making up 20% to 40% of cases.

Gallbladder Cancer

This cancer starts in the walls of the gallbladder. It is often discovered incidentally during procedures to investigate or remove gallstones.

Risk Factors for Biliary and Gallbladder Cancer

A risk factor is anything that increases the likelihood of developing cancer. Key risk factors include:

• Age: Most biliary cancer cases in India are diagnosed in people between 50 and 70 years of age.
• Ethnicity: In India, Indians have a higher likelihood of developing these cancers.

Medical Conditions

Certain pre-existing conditions can elevate the risk:

Primary Sclerosing Cholangitis (PSC), a progressive autoimmune disease causing scarring of the bile ducts.
Chronic liver diseases, including cirrhosis, bile duct stones, cholangitis, non-viral chronic liver disease, and polycystic liver disease.
Hepatitis B or Hepatitis C infection.
Inflammatory bowel diseases, such as Crohn's disease and Ulcerative colitis.
A history of bile duct cysts (choledochal cysts), which are prone to chronic inflammation.
Liver fluke infection (parasites more common in South and Southeast Asia, often due to consuming raw or undercooked fish).
Exposure to Thorotrast, a former medical contrast agent.

Lifestyle Factors

Smoking.
Excessive consumption of alcohol, particularly in those with alcohol-associated liver damage.

Family History

Certain genetic disorders, such as Lynch syndrome, may increase the risk of biliary tract cancers.

Symptoms

Biliary cancers are located deep within the body, which means signs and symptoms may not become apparent until the disease has reached a more advanced stage.

The most common symptoms include:

• Jaundice: A yellowing of the skin and the whites of the eyes. This is caused by high levels of bilirubin, a yellow pigment that builds up when the liver or bile ducts are malfunctioning.
• Itching: Excess bilirubin reaching the skin can cause persistent itchy skin.
• Unexplained weight loss or lack of appetite.
• Fatigue.
• Nausea and/or fever: Often resulting from abnormal liver function, infection due to bile duct blockage, or increased bilirubin.

• Light-coloured stools with dark-coloured urine: Bile is what gives stool its brown colour. If bile flow into the small intestine is obstructed, stools may become pale.
• Enlarged abdominal mass: If a bile duct tumour grows and interferes with nearby organs, a visible mass can be caused.
• Abdominal pain and/or bloating in advanced stages.

It is vital to note that having one or more of these symptoms does not definitively mean a person has biliary cancer. However, it is crucial to discuss these changes with a doctor for early diagnosis.

Diagnosis

Bile duct tumours are sometimes found accidentally during a CT scan or ultrasound for unrelated health concerns, or during routine gallbladder surgery. As early stages may be asymptomatic, only a small number of cases are diagnosed before the cancer has spread.

Tools and procedures for diagnosing biliary cancer include:

Blood Tests

While no single test can definitively detect the cancer, blood may be tested for elevated bilirubin levels or other substances to assess liver and gallbladder function. These tests help rule out other causes, such as infection. Doctors may also test for tumour markers like carbohydrate antigen 19-9 (CA 19-9) and carcinoembryonic antigen (CEA), though high levels of these do not confirm cancer and can indicate other conditions, such as bile duct obstruction.

Biopsy

A small tissue sample is surgically removed and examined under a microscope for cancer cells. Biopsy methods include:

Fine Needle Aspiration (FNA): A small needle is guided (often using CT scans) into the tumour to draw out a small sample of cells for review.
Endoscopic Retrograde Cholangiopancreatography (ERCP): A thin tube with a camera is passed through the mouth and digestive tract to view the bile ducts. A dye may be injected to make the ducts more visible on X-rays. This procedure can also be used to obtain tissue samples.

Imaging Tests

Used to identify abnormalities and determine the extent of cancer spread:

CT (Computed Axial Tomography) Scan: An X-ray technique that takes pictures from different angles to create a highly detailed image of the body.
MRI (Magnetic Resonance Imaging): Uses magnetic fields and radio waves to create detailed images of organs.
Magnetic Resonance Cholangiopancreatography (MRCP): A non-invasive alternative to ERCP that creates a 3D image of the bile ducts without the use of a dye.
Abdominal Ultrasound: Uses high-energy sound waves to create a picture of internal organs.

Staging

Each type of biliary cancer has its own staging system, typically sourced from bodies like the National Cancer Institute (NCI). Staging is crucial for determining the most appropriate treatment plan. The stages (0 to 4) describe how large the tumour is and how far the cancer has spread from its original site.

Treatment

SSCHRC has experts with significant experience in treating bile duct and gallbladder cancer using a personalised, multidisciplinary approach that includes surgery, radiation, and chemotherapy. Treatment decisions are based on the disease stage, the patient's physical condition, and the tumour's characteristics.

Surgical Treatment

Complete removal of the tumour is the most effective treatment for biliary cancer. This is often an option for patients with early-stage disease and good overall physical health.

Intrahepatic and Perihilar Tumours: Surgery usually requires the removal of portions of the liver (hepatectomy) and sometimes the main bile duct.
Distal Bile Duct Tumours: Because the bile duct passes through the pancreas in this area, surgery often requires the removal of parts of these organs, a procedure sometimes referred to as a Whipple procedure.
Bile Duct Reconstruction: If the main bile duct is removed during surgery, reconstruction is performed to re-establish bile flow from the liver to the intestine.
Portal Vein Embolization (PVE): If a significant portion of the liver must be removed, PVE may be used to block a blood vessel near the tumour. This process encourages the healthy, remaining part of the liver (the future liver remnant) to grow large enough and fast enough for safe tumour removal.

Management of Biliary Obstruction

Obstruction and infection of the bile ducts (cholangitis) can be life-threatening complications. Doctors can manage this by inserting a plastic or wire mesh tube called a stent into the bile duct to keep it open, correcting the obstruction and preventing infection.

Radiation Therapy

Radiation therapy uses focused, high-energy beams to destroy cancer cells. It can improve survival and offer a chance for prolonged disease control for patients who are not candidates for surgery. SSCHRC experts use advanced imaging and image guidance techniques (like CT or MRI scans) to safely deliver high doses of radiation to the tumour site while protecting nearby organs such as the stomach, small bowel, colon, and healthy parts of the liver.

Personalised radiation treatment plans may include:

Intensity Modulated Radiation Therapy (IMRT): Uses high-energy photons with controlled, shaped beams, often used for tumours near the gastrointestinal tract.
Stereotactic Body Radiation Therapy (SBRT): Delivers very high doses of radiation over one to two weeks, best suited for smaller tumours not near the gastrointestinal tract.
Proton Therapy: A radiation treatment that uses high-energy particles to target tumours while sparing nearby organs.
Radioembolization: Tiny pellets carrying a radiation source are injected into the arteries supplying the tumour, directly killing the cancer cells.

Systemic Therapies

Chemotherapy: Since many biliary cancers are discovered at an advanced stage when surgery is limited, chemotherapy is often used. It works by killing rapidly growing cells throughout the body, helping to control the spread of cancer, ease symptoms, and improve overall survival.
Targeted Therapy: This is a crucial area of research. Targeted therapies are designed to seek out and destroy specific genetic abnormalities or mutations within cancer cells. They are administered orally or via IV injection.
Clinical Trials: SSCHRC is committed to developing new treatments and clinical trials to enhance the prognosis and survival for biliary cancer patients. Trials may combine chemotherapy with surgery (before surgery to shrink tumours or after to reduce recurrence risk) or explore new targeted therapies for driving mutations like IDH, FGFR, and BRAF proteins.

Supportive Care

Biliary cancer and its treatments can lead to complications, including: stomach obstruction, delayed stomach emptying (gastroparesis), weight loss, malnutrition, fluid buildup in the abdomen (ascites), abdominal pain, chronic nausea/vomiting, and bile duct obstructions.

As a leading cancer centre, SSCHRC employs a multidisciplinary approach to manage these issues, offering services in supportive care, gastroenterology, interventional radiology, and pain management. Dedicated Survivorship Clinics also provide patients with follow-up care and support for life after cancer treatment.