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Treatment Options

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.

It is critical to seek a second opinion, particularly for an uncommon cancer like cholangiocarcinoma. A second opinion can provide more information and help you feel more confident about your chosen treatment plan.

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, 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 (stage III and IV) and in cases where the edges of the tissues removed at the operation show cancer cells (also called a positive margin).

Terminology Chart

Cholangiocarcinoma Terminology Chartclick to open

Surgery

Surgery

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. There are 2 general types of surgical treatment for cholangiocarcinoma — potentially curative surgery and palliative surgery.

 

A. Potentially curative surgery

This is used when imaging tests indicate a good chance that the surgeon may be able to remove all of the cancer. Doctors may use the term resectable to describe cancers they believe can be removed completely (by potentially curative surgery) and unresectable to describe those they think have spread too far or are in too difficult a place to be entirely removed by surgery. Unfortunately, only a small portion of bile duct cancers are resectable at the time they are first found.

For resectable cancers, the type of operation depends on the cancer location

  • Intrahepatic cholangiocarcinoma: These cancers are in the bile ducts within the liver. 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. Sometimes this means that a whole lobe (section) of the liver must be removed. This is called hepatic lobectomy. It is a complicated operation and requires an experienced team of surgeons and assistants. If the amount of liver tissue removed is not too great, the liver will function normally because its tissue has the ability to grow back.
  • 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.
  • 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. This operation is called a Whipple procedure, this is a complex procedure that requires an experienced surgical team.
  • Possible risks and side effects: The risks and side effects of surgery depend in large part on the extent of the operation and a person’s general health. All surgery carries some risk, including the possibility of bleeding, infections, complications from anesthesia, pneumonia, and even death in rare cases. People will have some pain from the incision for some time after the operation, but this can usually be controlled with medicine. Surgery for bile duct cancer is a major operation that might mean removing parts of several organs. This can significantly affect a person’s recovery and health after the surgery, because most of the organs involved in digestion, eating and nutrition problems are often long-term side effects of surgery for this cancer.

Liver transplantation for unresectable cancers: The American Association for the Study of Liver disease Guidelines recommends that potential transplant candidates should be expeditiously referred to major cancer centers that have established protocols for oncologic assessment and treatment approved by United Network for Organ Sharing (UNOS).

For some people with early stage unresectable intrahepatic or perihilar bile duct cancers, removing the liver and bile ducts and then transplanting a donor liver may be an option. In some cases, it might even cure the cancer.

For those who are eligible for a transplant there are some obstacles including:

  • Not many centers accept patients with bile duct cancer into their transplant programs.
  • Few livers are available for patients with cancer because they are generally used for more curable diseases.
  • Waiting until a liver is available can take a long for some people.

To overcome these obstacles, patients have two options:

  • One option is having a living donor (often a close relative) give a part of their liver for transplant. This can be successful, but it carries risks for the donor.
  • Another option is to treat the patient first with chemotherapy and radiation. This is followed by a transplant when a liver becomes available. This has been done as part of a clinical trial in the past, and may become a standard treatment for perihilar bile duct cancer in the future.

Possible risks and side effects: Like other surgeries for bile duct cancer, a liver transplant is a major operation with potential risks (bleeding, infection, complications from anesthesia, etc.). But there are also some additional risks after this surgery. People who get a liver transplant have to be given drugs to help suppress their immune system and prevent them from rejecting the new organ. These drugs have their own risks and side effects, especially the risk of getting serious infections. Some of the drugs used to prevent rejection can also cause high blood pressure, high cholesterol, and diabetes, can weaken the bones and kidneys, and can lead to the development of another cancer. After a liver transplant, regular blood tests are important to check for signs of rejection. Sometimes liver biopsies are also taken to see if rejection is occurring and if the anti-rejection medicines need to be changed.

 


 

B. Palliative surgery:

This may be performed to relieve symptoms or treat (or even prevent) complications, such as blockage of the bile ducts. This type of surgery is performed when the tumor is too widespread to be completely removed. Palliative surgery is not expected to cure the cancer, but it can sometimes help someone feel better and sometimes can even help them live longer. Palliative surgery includes biliary bypass or inserting biliary stent

  • Biliary bypass: In some cases, a doctor may think that a cancer is resectable based on the information available (imaging tests, laparoscopy, etc.), but once surgery is started it becomes clear that the cancer is too advanced to be removed completely. At this point the surgeon may do a biliary bypass to allow the bile to flow into the intestines to reduce symptoms such as jaundice or itching. In this palliative procedure, the surgeon creates a bypass around the tumor blocking the bile duct by connecting part of the bile duct before the blockage with a part of the duct that lies past the blockage. Often, the gallbladder is used to provide some of the bypass.
  • Biliary stent or biliary catheter: If cancer is blocking the bile duct, the doctor may insert a small tube (called a stentor catheter) into the duct to help keep it open. This may be done as part of a cholangiography procedure such as percutaneous transhepatic cholangiography (PTC) or endoscopic retrograde cholangio-pancreatography (ERCP) or, in some cases, during surgery. A stent opens the duct to allow the bile to drain into the small intestine, while a catheter drains into a bag outside the body that can be emptied when needed. The stent or catheter may need to be replaced every few months if it becomes clogged and to reduce the risk of infection and gallbladder inflammation.
Radiation
Locoregional Therapy
Systemic Chemotherapy
Targeted Therapy
Immunotherapy
Palliative Therapy

Making treatment decisions

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. In choosing a treatment plan, there are some factors to consider:

The location and extent of the cancer

Whether the cancer is resectable (removable by surgery)

The likely side effects of treatment

Your overall health

The chances of curing the disease, extending life, or relieving symptoms

It is critical to seek a second opinion, particularly for an uncommon cancer like cholangiocarcinoma. A second opinion can provide more information and help you feel more confident about your chosen treatment plan.

*adapted from cancer.net

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