From the Guidelines
Cholangiocarcinoma treatment should prioritize surgical resection when possible, as it offers the best chance for cure, with liver transplantation (LT) being an effective option for perihilar cholangiocarcinoma in highly selected patients, achieving a 65% rate of disease-free 5-year survival 1.
Classification and Risk Factors
Cholangiocarcinoma is a malignant tumor arising from the epithelium of the bile ducts, accounting for about 3% of all gastrointestinal tumors and is the second most common primary liver tumor 1. It can be classified based on location as intrahepatic (within the liver), perihilar (at the junction of the main bile ducts), or distal (in the portion of the bile duct outside the liver). Risk factors include primary sclerosing cholangitis, liver fluke infections, biliary stones, hepatitis B or C, cirrhosis, and certain genetic conditions.
Symptoms and Diagnosis
Symptoms often appear late and may include jaundice, abdominal pain, weight loss, fatigue, and clay-colored stools. Diagnosis typically involves blood tests, imaging studies like CT, MRI, or MRCP, and often requires tissue biopsy for confirmation.
Treatment Options
Treatment depends on the stage and location but generally includes:
- Surgical resection when possible, which offers the best chance for cure
- Chemotherapy (typically gemcitabine plus cisplatin) for unresectable disease
- Radiation therapy
- Targeted therapies based on genetic testing
- Immunotherapy in select cases
- Palliative procedures like stenting may be needed to relieve biliary obstruction
Prognosis
Prognosis remains challenging with 5-year survival rates ranging from 15-30%, highlighting the importance of early detection and multidisciplinary care at specialized centers 1. Liver transplantation can be effective for perihilar cholangiocarcinoma, with a protocol combining neoadjuvant chemoradiation and LT leading to significantly lower recurrence rates and higher long-term survival rates than other existing treatment modalities 1.
From the Research
Definition and Classification of Cholangiocarcinoma
- Cholangiocarcinoma is a rare biliary adenocarcinoma associated with poor outcomes, subdivided into extrahepatic and intrahepatic variants 2.
- Intrahepatic cholangiocarcinoma is further differentiated into peripheral mass-forming tumors and central periductal infiltrating tumors 2.
Treatment Options for Cholangiocarcinoma
- Surgery is the main therapeutic option for cholangiocarcinoma, with surgically resectable disease being the only curable treatment option 3, 2.
- Chemotherapy, such as gemcitabine plus cisplatin, is recommended as first-line treatment for advanced cholangiocarcinoma 3, 4.
- Targeted radiation therapy may also be considered as part of a multispecialty treatment approach 2.
Prognosis and Survival Rates
- The 5-year survival rate for cholangiocarcinoma is 5%-10%, with 5-year survival rates of 25%-30% reported in cases with potentially curative surgery 3.
- Gemcitabine plus cisplatin chemotherapy has been shown to prolong survival in patients with unresectable hilar cholangiocarcinoma, with a median overall survival of 12.8 months 4.
Role of Gemcitabine in Treatment
- Gemcitabine has demonstrated response in similar cancers and may be a reasonable alternative to best supportive care for patients not considered candidates for surgery 5.
- Gemcitabine plus cisplatin may be a safe and feasible preoperative treatment in initially unresectable locally advanced or borderline resectable cholangiocarcinoma 6.