Cholangiocarcinoma (Bile Duct Cancer) and Gallbladder Cancer

The liver produces bile, a digestive juice that is carried from the liver to the intestine by a system of passages called the bile ducts. Tiny bile ducts spread out through the liver to collect the bile and they come together just outside the liver to form the common (main) duct; the gallbladder is a pouch off of the side of the common duct where bile is stored between meals.

Cancers called cholangiocarcinoma can develop at any point along the path of the bile ducts. Tumors that arise from the small branches inside liver grow into and are called intrahepatic cholangiocarcinoma. These tumors are often quite large when discovered since they usually form in people without any known liver disease who have no reason to be looking for them with routine screening, Tumors that start in larger ducts outside the liver near the area where the ducts from the right and left sides of the liver join to form the common duct are called hilar cholangiocarcinomas and commonly cause narrowing of the ducts, leading to jaundice (yellowing of the skin and eyes) due to backing-up of bile into the blood; removing these tumors almost always requires removing part of the liver along with the bile ducts. Tumors that form in the lower part of the duct near where it passes through the pancreas and enters into the intestine are called distal cholangiocarcinomas. They also lead to jaundice, and removing them usually requires removal of the head of the pancreas and the first part of the intestine (duodenum), a procedure commonly referred to as a Whipple procedure (after Allen Whipple, the first surgeon to describe the procedure).

Treatments for Bile Duct Cancer and Gallbladder Cancer

Like HCC, the incidence of cholangiocarcinoma is rising in the U.S. Cholangiocarcinoma and gallbladder cancer are among the most challenging tumors for liver surgeons because they are often large and involve nearby blood vessels, and may require new connections to be made between the intestine and the bile ducts in the liver. Surgery for cholangiocarcinoma has been a special interest of our surgical team at Mount Sinai for many years. Unlike HCC, chemotherapy has an important role in treating cholangiocarcinoma, and a coordinated plan including neoadjuvant (before surgery) and/or adjuvant (after surgery) chemotherapy is often the best way to achieve long-term survival. Cholangiocarcinoma has often spread to nearby lymph nodes by the time it is discovered. This doesn’t mean that we can’t do surgery, but when the lymph nodes contain cancer we know that the chances of the cancer coming back in other near-by nodes is high. It is also not unusual that, despite removing as much as is technically possible, there are still microscopic cancer cells at the margin (edge) where we cut through the tissues. When there is cancer in the lymph nodes or at the margins, treating the area with radiation after recovery from surgery is another way to reduce the chances of cancer coming back.

For hilar cholangiocarcinoma that cannot be removed by resection, especially cases that develop in people with primary sclerosing cholangitis (an autoimmune disease that causes inflammation of the bile ducts leading to the development of multiple narrowed areas called strictures), Mount Sinai is one of the few select centers in the US with a liver transplant protocol that has been approved by UNOS (the organization that determines who gets organs for transplantation), enabling us to prioritize our patients on the transplant waiting list.