Please ensure that you are familiar with the CCA Patient Toolkit before reading the page content
Please ensure that you are familiar with the CCA Patient Toolkit before reading the page content

Cholangio 101


Primary Locations (origin) defines the subtype of CCA


Cholangiocarcinoma is the clinical name for Bile Duct Cancer.
The easiest way to pronounce this name is to first break it in half, ie:  Cholangio + carcinoma
Chol – angio + car – cinoma

  • Chol’e means bile  +
  • angio means vessel – ie Bile Duct tube +
  • carcinoma means a type of cancer that grows in the tissue lining the body’s organs
Cholangiocarcinoma is a cancer that begins in the bile ducts.

Cholangiocarcinoma is the clinical name for bile duct carcinoma.

The bile duct system is like a network of bile streams called ductules originating in the liver and then feeding down to form two larger ducts. Think of two networks of streams coming together – a right network and a left network.

These two ducts are called the “Left” and “Right” “Hepatic Ducts” which join together and form one common bile duct. This area is referred to as the “Hilar region” or more recently the “Perihilar” region. (Hilar means slit or opening)

The “Common bile duct” tube is about 125 -150 mm long, extending from the liver to the small intestine called the Duodenum. The bile duct’s function is to move a fluid called bile from the liver and gallbladder to the small intestine, where it helps digest the fats in food.

About one-third of the way down the common bile duct, the gallbladder (an organ that stores bile) attaches by a small duct called the cystic duct.

The common bile duct then passes down through part of the pancreas before it empties into the first part of the small intestine (the duodenum), next to where the pancreatic duct also enters the small Duodenum.

Primary Tumours

(Means location where the cancer begins)

Primary Tumours can develop in any part of the bile duct and are named after their exact location –
Click each type below to see more

  1. iCCA: Intrahepatic cholangiocarcinoma
  2. pCCA: Perihilar cholangiocarcinoma
  3. dCCA: Distal cholangiocarcinoma

Intrahepatic Cholangiocarcinoma
Intrahepatic CCA occurs inside the liver where cancer develops in the hepatic bile ducts or the smaller intrahepatic biliary ducts. In some cases, patients express a combined diagnosis.

Perihilar (Hilar or Klatskin Tumor) Cholangiocarcinoma
These cancers develop where the right and left hepatic ducts have joined and are leaving the liver. These are the most common type of cholangiocarcinoma accounting for more than half of all bile duct cancers.

Distal Cholangiocarcinoma
Distal CCA occurs outside the liver after the right and left hepatic bile ducts have joined to form the common bile duct. This type of cancer is found where the common bile duct passes through the pancreas and into the small intestine.

Extrahepatic Cholangiocarcinoma
Because perihilar and distal bile duct cancers start outside the liver, they are often grouped together and referred to as extrahepatic cholangiocarcinoma.

Cholangiocarcinoma can also be divided into types based on how the cancer cells look under the microscope.

More than 95% of bile duct cancers are carcinomas and most are adenocarcinomas.

Adenocarcinomas are cancer that begins in the glandular (secretory) cells. Glandular cells are found in epithelial tissue (Skin layer) that lines and protects internal organs. These glandular cells make and release substances such as mucus, digestive juices, or other fluids to protect the skin layer.

Simple Explanation

Firstline treatment means the medically accepted and practiced first approach. If unsuccessful a second-line approach will be looked at…etc.

Surgery if possible
  • Surgery is a potential Curative intervention
  • Only a very small percentage qualify for surgery, due to most diagnoses are late-stage and metastatic.
  • Curative Surgery Outcomes are also very small with most patients relapse.
  • Chemotherapy is described as a management tool
  • Chemotherapy is not a curative treatment. (Surgery potentially is)
  • The primary objective is to slow or stop the progression
  • Some patients see overall regression
  • Some Patients see all visible cancer activity eliminated and are referred to as NED (No Evidence of Disease)
  • Typical First-line Chemotherapy is Gemcitabine and Cisplatin (Gem/Cis)
  • Typical First-line treatment duration is 6 months
  • Treatment cycles are 2 weeks on – 1 week off
  • Scans measure the treatment’s progress and are typically 9 to 12 Weekly depending on the aggression.
  • Chemotherapy Variations: There are many combinations of different chemos.
  • Chemotherapies are also combining with newer immunotherapy drugs. The basic expectation is that the chemotherapy agent will knock down and or weaken a tumour’s defenses, which creates a better (more robust) pathway, thus allow the immunotherapy drug to have a greater chance of success.
  • For Immunotherapy – see “Immuno 101
  • Note Immunotherapy treatments are not considered first-line treatments for Cholangiocarcinoma – but you should definitely discusses this with your medical team.
Symptoms appear late

Sudden onset of abdominal pain, Fatigue, then jaundice, and weight loss. These symptoms are similar to many other ailments in the beginning and therefore often dismissed.

There is no current early detection method for CCA.

It is unfortunate that most patients will feel no ill effects until the cancer is very advanced. This is one of Cholangiocarcinoma’s biggest threats, and contributes to many being excluded from potentially curative surgery.

Most Common Symptoms
  • Chills
  • Clay-colored stools
  • Fever
  • Itching
  • Loss of appetite
  • Weight loss
  • Pain in the upper right abdomen that may radiate to the back
  • Yellowing of the skin

Staging is a way of describing a cancer, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer’s stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient’s prognosis (chance of recovery). There are different stage descriptions for different types of cancer.

One tool that doctors use to describe the stage is the TNM system. This system uses three criteria to judge the stage of the cancer: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to the rest of the body. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the cancer so doctors can work together to plan the best treatments.

TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:

How large is the primary tumor and where is it located? (Tumor, T)
Has the tumor spread to the lymph nodes? (Node, N)
Has the cancer metastasized (spread) to other parts of the body? (Metastasis, M)
Intrahepatic Cholangiocarcinoma (cancer that occurs in the bile duct within the liver) is staged using the same system as liver cancer. The staging of both Intrahepatic and Extrahepatic Cholangiocarcinoma is below.

Basic Staging Descriptions

  1. Grade 0: The cancer is only growing in the innermost layer of the bile duct and has not spread to lymph nodes or distant sites.
  2. Grade 1: The cancer has grown into deeper layers of the bile duct wall but it has not grown all the way through the wall. It has not spread to lymph nodes or distant sites.
  3. Grade 2: The cancer is either a single tumor that has grown into a blood vessel (T2a) or there are multiple tumors (T2b). The cancer has not grown into any nearby organs or structures. It has not spread to nearby lymph nodes (N0) or distant sites (M0).
  4. Grade 3: The cancer has grown into nearby structures such as the duodenum (first part of the small intestine), colon, stomach, abdominal wall, diaphragm, or lymph nodes around the portal vein. It has not spread to nearby lymph nodes or distant sites.
  5. Grade 4: The cancer has spread (metastasised) to other organs away from the original primary site.

Australia Cases per yr: Sub 1,000 – est 900
USA-North America: Sub 10,000 – est 9,000
UK and Europe: (to beupdated)

Overall Average Survival Period = 6.8 months
Via Cholangiocarcinoma Foundation
The incidence and mortality of Cholangiocarcinoma in North America and Europe have increased dramatically in the past few decades.In the U.S., there are now between 5,000 and 10,000 new cases diagnosed each year.

Rates are highest among Hispanics and Asians, and men appear to have slightly greater mortality from the disease than women.

The highest incidence rates are in Eastern and South-Eastern Asia, with a peak in Thailand.

The prognosis for bile duct cancer

5 Year Survival Rates 

Seers all stages

  • Intrahepatic: 8%
  • Extrahepatic 10%
  • Metastatic – Intra or Extra: 1 %
  • Overall

See Source Link for all variations

The American Cancer Society relies on information from the Surveillance, Epidemiology, and End Results (SEER) database, maintained by the National Cancer Institute (NCI), to provide survival statistics for different types of cancer.

How do cancers form
What are the common categories of cancer?

Emerging Treatment Strategies for Advanced Cholangiocarcinoma
by Lipika Goyle, MD, Massachusetts General Hospital 2021
View Speaker Slides | Speaker Notes 

Dr. Thomas Fishbein 2020
Hilar Cholangiocarcinoma diagnosis (Extrahepatic)

Dr. Kate Kelly 2016

Dr. Kate Kelly 2017

Mayo Clinic Radio 2019

Good resources for terminologies
What is Primary Cancer?

This is the original tumour location. A tumour in the bile duct is called a Cholangiocarcinoma if it spreads (Metastasises) to other parts of the body ie. Lungs, it is still Cholangiocarcinoma, not lung cancer, etc.

What is Metastasis?

When the cancer spreads to other locations away from its primary tumour location ie. Lungs, Liver, etc.

What is a Mutation?

Any change in the DNA sequence of a cell. Mutations may be caused by mistakes during cell division, or they may be caused by exposure to DNA-damaging agents in the environment.

What are the Curative Options?
  • Surgical operations are the only known Curative action, but stats are very poor
  • More recently immunotherapy has been seen as a curative option
What are the Surgery Procedures?
  • ECRP: Surgical, typically investigative same-day procedure via the mouth
  • Whipple: Surgical, a significant operation typically removing all or part of multiple sites such as gallbladder, head of the pancreas, and stomach
  • Resection: Surgical, typically refers to Intrahepatic patients, removing a portion of the liver and gallbladder. This can also refer to removing a section of the bile duct for Extrahepatic patients which often include the gallbladder.
What is Chemotherapy?
  • A drug that is infused or oral
  • Typically the first line action is Gem/Cis – Gemcitabine & Cisplatin
  • Chemotherapy is not regarded as curative, but the best management tool available
  • Chemotherapy is an infusion process carried out in the Chemo wards of most hospitals, typically this will take 3 – 5 hours. Some Chemotherapy options such as Capecitabine are taken orally

Most treatment programs are 3 to 6 months in length and can be repeated several times.

What is the CA 19- 9 Marker?

A measure in a blood test (carbohydrate antigen) – a tumor marker that is observed in elevated serum concentration with metastatic GI cancers. This is not always an accurate measurement.

What are Clinical Trials?

There are many trials coming available in both immunotherapy and combination immunotherapy and Chemotherapy. Most patients embrace trials as an opportunity to move forward.

What is Immunology?

Immunology is a fast-evolving knowledge pool of using the body’s immune system to defeat cancer. Immunotherapy is the specifically designed option derived from the immunology pool that is applied to the patient. This is worth reading up about …[continue]

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