Cholangiocarcinoma (pronounced koh-lan-jee-oh-kar-sin-OH-muh) is a cancer that begins in the bile ducts. The bile ducts are a network of tubes that carry bile from the liver and gallbladder to the small intestine. Bile helps the body digest fats.Cholangiocarcinoma is also commonly called bile duct cancer.There are three general locations where it can begin:Intrahepatic: inside the liverPerihilar: where the right and left bile ducts leave the liverDistal: farther down the bile duct, closer to the small intestineWhere the cancer begins can affect the symptoms, treatment options, and prognosis.

Cholangiocarcinoma is considered a rare cancer in the United States.The American Cancer Society estimates that approximately 8,000 people in the United States are diagnosed with bile duct cancer each year. The actual number may be higher because these cancers can sometimes be difficult to diagnose or may be classified differently. That means many people diagnosed with cholangiocarcinoma find themselves learning about a disease they had never heard of before.

Symptoms can vary depending on where the cancer is located and how advanced it is. Possible symptoms include:Yellowing of the skin or whites of the eyes (jaundice)Dark-colored urinePale or clay-colored stoolsItchy skinAbdominal pain or discomfortUnexplained weight lossFeverNausea or vomitingFatigueThese symptoms can have many causes other than cancer. Having one or more of them does not necessarily mean someone has cholangiocarcinoma. However, persistent or unexplained symptoms should be discussed with a healthcare professional.

In many cases, doctors cannot identify one specific cause. Certain medical conditions and circumstances can increase the risk, including:Primary sclerosing cholangitis (PSC)Certain abnormalities of the bile ductsSome chronic liver conditionsCertain parasitic infections, particularly liver fluke infections that are more common in parts of Southeast AsiaHaving a risk factor does not mean that a person will develop cholangiocarcinoma, and many people diagnosed with the disease do not have an obvious risk factor.

At this time, there is no routine screening test for cholangiocarcinoma recommended for the general population.Because the bile ducts are deep inside the body and early disease may not cause obvious symptoms, the cancer can be difficult to detect at an early stage.People with certain medical conditions that increase their risk may have different monitoring recommendations, so this is something to discuss with a physician.

Doctors may use several different tests to diagnose cholangiocarcinoma and determine how far it has progressed. These can include:Blood testsUltrasoundCT scansMRI/MRCPEndoscopic procedures such as ERCPBiopsy or other tissue samplingTumor-marker testing, including CA 19-9 in appropriate circumstances No single test tells the entire story. Doctors generally combine test results with the patient's symptoms, medical history, imaging, and other information to determine the diagnosis and treatment plan.

For some patients, yes.When a tumor can be completely removed, surgery may be performed with curative intent.The type of surgery depends on where the cancer is located. It may involve removing part of the bile duct, part of the liver, or other nearby structures.Surgery for cholangiocarcinoma can be complex, so patients may want to discuss their case with a medical team that has experience treating this rare cancer.

Treatment depends on many factors, including where the cancer began, whether it has spread, whether it can be removed surgically, the characteristics of the tumor, and the patient's overall health. Treatment may include:SurgeryChemotherapyImmunotherapyTargeted therapyRadiation therapyBiliary stents or drainage proceduresClinical trialsSometimes several treatments are used together. The National Cancer Institute notes that treatment differs substantially between resectable and unresectable disease.

For some patients, yes.When a tumor can be completely removed, surgery may be performed with curative intent.The type of surgery depends on where the cancer is located. It may involve removing part of the bile duct, part of the liver, or other nearby structures.Surgery for cholangiocarcinoma can be complex, so patients may want to discuss their case with a medical team that has experience treating this rare cancer.

This is an important word to understand.Resectable means that doctors believe the cancer may be able to be completely removed with surgery.Unresectable means that the cancer cannot be completely removed with surgery, which can happen because of its location, how extensively it has spread, or other medical considerations.Whether a tumor is resectable can have a major impact on the treatment plan.

Chemotherapy uses medicines that kill cancer cells or slow their growth.For advanced or unresectable bile duct cancer, chemotherapy regimens may include drugs such as gemcitabine and cisplatin.Treatment has continued to evolve, and chemotherapy may be combined with immunotherapy or followed by other treatments depending on the individual situation.

Immunotherapy is a type of cancer treatment that helps the body's immune system recognize and attack cancer cells.For some people with advanced cholangiocarcinoma, immunotherapy medicines such as durvalumab or pembrolizumab may be used in combination with chemotherapy.Whether immunotherapy is appropriate depends on the individual's cancer and overall medical circumstances.

Cancer cells sometimes contain specific genetic changes that help them grow.Targeted therapies are designed to act on particular characteristics of cancer cells.This is one reason doctors may recommend molecular or genomic testing of a tumor, particularly when cholangiocarcinoma is advanced. Not every patient will have a genetic change that can be treated with a targeted drug, but testing may identify treatment options or clinical trials that otherwise might not be considered.

This is one of the hardest questions for patients and families. There is no single prognosis for everyone with cholangiocarcinoma. Outcomes depend on many factors, including:Where the cancer beganHow early it was detectedWhether it has spreadWhether it can be completely removedThe characteristics of the tumorThe person's overall healthHow the cancer responds to treatment Statistics describe what happened to groups of people in the past. They cannot tell one person exactly what will happen to them.

One reason is that there is no routine screening test for the general population.Early symptoms can also be subtle or absent, and symptoms such as fatigue, abdominal discomfort, or digestive changes can have many different causes.By the time some patients develop more recognizable symptoms, such as jaundice, the disease may already be more advanced.

A second opinion can be a reasonable thing to consider after a cancer diagnosis, particularly when the disease is rare or the recommended treatment is complex.A second opinion may provide additional information about:The diagnosisThe stage of the cancerWhether surgery is possibleTreatment optionsMolecular testingClinical trials Getting another medical opinion does not necessarily mean disagreeing with the first doctor. It can simply give a patient and family more information before making an important decision.

It can be difficult to remember everything during an appointment. Consider bringing a written list of questions. Some questions might include:Where exactly is my cancer located?What type of cholangiocarcinoma do I have?What stage is it?Has it spread?Is it resectable?Is surgery an option?Should I have molecular or genomic testing?What treatment options are available?What is the goal of each treatment?What side effects should I expect?Are there clinical trials I should consider?Would a second opinion be helpful?Should I be treated at a cancer center with experience in cholangiocarcinoma?What symptoms should cause me to call my medical team?What can be done to manage pain, nausea, itching, fatigue, or other symptoms? And perhaps one of the most important questions:"If this were your family member, what questions would you want answered before making a treatment decision?"

Because cholangiocarcinoma is rare, finding reliable information can make a tremendous difference. Some organizations that provide information and support include:National Cancer Institute (NCI)American Cancer SocietyCholangiocarcinoma FoundationCraine’s Cholangiocarcinoma CrewThese resources can provide additional information about the disease, treatment, research, and support for patients and families.

My book, Everything to Stand, tells the story of my own journey with cholangiocarcinoma — the diagnosis, the treatment, the uncertainty, the people who stood beside me, and the faith that carried me through it. My experience is my experience. Another person's journey may look completely different.But if sharing my story helps even one patient or family feel less alone, ask another question, seek another opinion, find a source of encouragement, or simply get through another difficult day, then telling the story is worthwhile. Don't lose hope. Keep asking questions. Keep moving forward. And remember that you don't have to walk the journey alone.*This page is intended for general educational purposes and is not a substitute for professional medical advice, diagnosis, or treatment. Medical information changes as research progresses. Anyone facing a cholangiocarcinoma diagnosis should discuss their individual circumstances and treatment options with their healthcare team.