MUSC Health Liver Transplant Program to Include Unresectable Cholangiocarcinoma Patients

Jared White, M.D.

MUSC Health abdominal transplant surgeons have expanded its liver transplant program to include patients diagnosed with hilar cholangiocarcinoma, offering hope to individuals diagnosed with this complex and often deadly disease.

In 2020, Jared White, M.D., surgical director of MUSC Health’s liver transplant program, and his team initiated a protocol for the program to include unresectable cholangiocarcinoma as an indication for liver transplantation.

Established protocols, particularly from the Mayo Clinic, and numerous studies have shown improved survival and encouraging results compared to surgical resection attempts or other standards of care for cholangiocarcinoma.

“Five-year survival following surgical resection attempts for cholangiocarcinoma falls in the 20-40% range based on numerous studies with as much as 75% disease recurrence rates,” White says. “With unresectable cholangiocarcinoma, five-year survival without liver transplantation approaches zero. Data from the Mayo Clinic has shown that in well-selected patients with unresectable cholangiocarcinoma the one-year overall survival rate for patients who undergo liver transplant is more than 90% after one year and 82% at five years.”

Hilar cholangiocarcinoma, also known as Klatskin’s tumor, begins in the liver hilum, where the right and left bile ducts join to form the common bile duct just proximal to the gallbladder. Although it is a relatively rare malignancy – less than 2% of all cancers nationwide – the morbidity and mortality for this disease is high -- with a life expectancy often six months or less according to White.

“We want to encourage physicians throughout the state to send patients with concern for cholangiocarcinoma to our group so we can evaluate them, treat them and get them listed for liver transplant when indicated,” says White, who has extensive experience performing complex hepatobiliary surgical procedures such as liver transplant, repair of bile duct injuries and liver cancers, including those that require vascular resection and reconstruction.

As the only transplant center in South Carolina, MUSC has the capability and the expertise of a multidisciplinary team that includes liver transplant surgeons, hepatologists, hematologists and oncologists, advanced endoscopists, radiation oncologists and interventional and diagnostic radiologists to evaluate and treat these complex patients in addition to following pre- and post-operative treatment protocols and outcomes. The team also evaluates cases through a multi-disciplinary virtual liver tumor board.

Patients must be young enough – typically in the 40 to 60 age range – and strong enough to withstand surgery and recovery. They must also be lymph node negative and not have undergone a needle biopsy, and the tumor must not exceed 3 centimeters.

“Percutaneous needle biopsy runs the risk of dispersing the cancer cells, increasing the risk of cancer recurrence, and makes the patient ineligible for transplant,” White says. “We encourage our referring providers to contact our program regarding the workup and management of these patients to ensure that liver transplantation remains a possible option before embarking on any therapies that might preclude the safety of liver transplant.”

Diagnosing cholangiocarcinoma is most commonly made through a combination of blood tests, 3-dimensional imaging such as CT or MRI and intraductal biopsy using ERCP and lab testing using fluorescence in situ hybridization (FISH). Despite all of these methods, diagnosing cholangiocarcinoma definitively can be quite challenging due to the nature of the disease, which tends to grow along the surface of the bile ducts and presents at advanced stages when the biliary tree is completely obstructed.

According to nationally utilized protocols, patients being considered for liver transplant for cholangiocarcinoma must undergo neoadjuvant therapy, which is a combination of chemotherapy and external beam radiation therapy (EBRT). EBRT consists of 25 to 30 fractions of high-dose radiation once or twice daily for as many as three to six weeks. Additionally, patients receive and remain on chemotherapy, an oral agent known as capecitabine, during the radiation treatments and until transplantation. After completing neoadjuvant therapy, patients must be restaged with CT or MRI and endoscopic lymph node sampling to ensure no progression of disease or evidence of metastatic disease prior to being listed for transplantation.

The surgical procedure is more challenging than liver transplantation for other indications because of the radiation therapy according to White. “Radiation makes the removal and preservation of the blood vessels more challenging. We resect the bile duct completely, and occasionally these patients need vascular conduits if we can’t preserve the blood vessels due to the changes from radiation. We bring up a loop of intestines to sew to the donor bile duct. Overall, the end result is very similar to any other liver transplant.”

White says for the state of South Carolina and surrounding regions, cholangiocarcinoma is a complex disease and associated with extremely high morbidity and mortality, with limited curative options outside of complete resection or liver transplantation. “If there is any potential for transplant or resection, we encourage physicians whose patients have obstructive jaundice or an indication for cholangiocarcinoma to refer them to MUSC Health.”

For more information on MUSC Health’s transplant programs or to refer a patient for transplant evaluation, please call 843-792-5097.