From the Guidelines
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. This type of cancer can be divided into intrahepatic cholangiocarcinoma (iCC), perihilar cholangiocarcinoma (pCC), and distal cholangiocarcinoma (dCC), with pCC being the most common type, accounting for 50-60% of cases 1. The prognosis of cholangiocarcinoma is generally poor due to the difficulty of early diagnosis and limited treatment methods, with patients having a median survival of 24 months after initial diagnosis 1.
Key Characteristics of Cholangiocarcinoma
- It is a rare and aggressive cancer that develops in the bile ducts
- Can occur in bile ducts inside the liver (intrahepatic), at the junction where ducts exit the liver (perihilar), or in ducts outside the liver (distal)
- Risk factors include primary sclerosing cholangitis, liver fluke infections, bile duct stones, and certain liver diseases
- Symptoms typically appear late and include jaundice, abdominal pain, weight loss, fatigue, and clay-colored stools
- Diagnosis usually involves blood tests, imaging studies like CT or MRI, and often a biopsy
Treatment Options
- Treatment depends on the cancer's location and stage but may include surgery to remove the tumor, chemotherapy (commonly gemcitabine and cisplatin), radiation therapy, or targeted therapies
- Complete resection is the main curative therapy for patients with extrahepatic cholangiocarcinoma, with surgical procedures based on the portion of the extrahepatic biliary tree in which the lesion resides 1
- Liver transplantation is the only other potentially curative option for patients with extrahepatic cholangiocarcinoma, recommended for highly selected patients with either unresectable disease or underlying chronic liver disease precluding surgery 1
Importance of Early Detection
- Early detection through regular medical check-ups, especially for those with risk factors, is crucial for improving outcomes
- Regular medical check-ups and awareness of risk factors are essential for the early detection and management of cholangiocarcinoma, potentially improving morbidity, mortality, and quality of life.
From the Research
Definition and Classification of Colangiocarcinoma
- Colangiocarcinoma is a rare biliary adenocarcinoma associated with poor outcomes 2.
- It is subdivided into extrahepatic and intrahepatic variants, with intrahepatic cholangiocarcinoma further differentiated into peripheral mass-forming tumors and central periductal infiltrating tumors 2.
- Cholangiocarcinoma can be classified into subtypes based on location: intrahepatic, perihilar, and distal (extrahepatic) 3, 4.
Characteristics and Risk Factors
- Cholangiocarcinoma typically forms in the setting of cholestasis and chronic inflammation 2.
- Most patients do not carry the documented risk factors, which include infections and inflammatory conditions 2.
- The incidence of cholangiocarcinoma has seen a significant increase over the last several decades 2.
Diagnosis and Treatment
- Surgical resection is the only curable treatment option, which may involve liver transplantation in certain selected cases 2, 5, 6.
- Management strategies include multispecialty treatments, with consideration of systemic chemotherapy, and targeted radiation therapy 2, 5, 6.
- Neoadjuvant therapy represents an appealing approach to improve local and distant control, achieve R0 resection, and prevent distant metastasis 3.
- Chemotherapy with gemcitabine and cisplatin is considered the standard for palliative care 6.