From the Guidelines
Cholangiocarcinoma typically presents with jaundice, weight loss, abdominal pain, nausea, vomiting, and fever, with the most common symptoms being jaundice (84-90%) and weight loss (35%). The presentation of cholangiocarcinoma can vary depending on the location of the tumor, with distal tumors causing earlier jaundice while hilar or intrahepatic tumors may remain asymptomatic until advanced stages 1.
Common Symptoms
- Jaundice (84-90%)
- Weight loss (35%)
- Abdominal pain (30%)
- Nausea and vomiting (12-25%)
- Fever (10%)
Diagnosis
Diagnosis of cholangiocarcinoma is often challenging due to the nonspecific nature of symptoms and the anatomical location of bile ducts, which is why it is often diagnosed at advanced stages 1.
Laboratory Findings
Laboratory findings typically show elevated liver enzymes, particularly alkaline phosphatase and gamma-glutamyl transferase, along with increased bilirubin levels 1.
Imaging Modalities
Imaging modalities such as abdominal ultrasound, multidetector computed tomography (MDCT), and magnetic resonance cholangiopancreatography (MRCP) are used to evaluate populations suspected of having cholangiocarcinoma, with MDCT and MRCP being the main imaging modalities for diagnosis and staging of the disease 1.
Tumor Markers
Tumor markers such as CA19-9 and CEA can support a diagnosis of cholangiocarcinoma, but are not specific for the disease 1.
Risk Factors
Risk factors for cholangiocarcinoma include primary sclerosing cholangitis, liver fluke infections, biliary tract stones, and certain congenital abnormalities of the bile ducts 1.
Recent Guidelines
Recent guidelines, such as the EASL-ILCA clinical practice guidelines on the management of intrahepatic cholangiocarcinoma, emphasize the importance of early diagnosis, molecular characterization, accurate staging, and personalized multidisciplinary treatments for the management of cholangiocarcinoma 1.
From the Research
Presentation of Cholangiocarcinoma
Cholangiocarcinoma, a tumor of the bile duct epithelium, can present in various ways depending on its anatomical location. The three main categories are perihilar, intrahepatic, and extrahepatic.
- Patients with cholangiocarcinoma often complain of:
- Intrahepatic cholangiocarcinomas usually present as mass lesions that are asymptomatic or cause nonspecific systemic symptoms such as fatigue, fever, and weight loss 3
- Hilar and extrahepatic tumors most commonly present with jaundice, though cholangitis also can be seen 3
- Obstructive jaundice is a major clinical sign of cholangiocarcinomas, which is persistent and progressive 4
- Biological tumor markers such as carbohydrate antigen 19-9 and carcinoembryonic antigen can be useful in diagnosing cholangiocarcinoma in some patients, but these are nonspecific and may be elevated in infection, inflammation, or any obstruction 2, 3, 4, 5
Diagnostic Modalities
- Imaging modalities, including transabdominal ultrasound, computed tomography, and magnetic resonance imaging, play an important role in detecting tumors as well as guiding biopsy procedures and staging workups in cholangiocarcinoma 2
- Endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP) are recommended as the next step in the evaluation of extrahepatic cholangiocarcinoma 2
- ERCP has been used for the diagnosis and management of cholangiocarcinoma for many years 3
- MRCP-magnetic resonance cholangiopancreatography and virtual endoscopy are more helpful methods in the diagnostics of cholangiocarcinoma 5
- FDG positron emission tomography has been suggested to be a sensitive technique in identifying small bile duct cancers 5
Risk Factors and Incidence
- Primary sclerosing cholangitis (PSC) is a major risk factor for cholangiocarcinoma, and the incidence of cholangiocarcinoma in PSC patients is nearly 10 to 1000 times higher than that in the general population 6
- Cumulative 5-year, 10-year, and lifetime incidences of cholangiocarcinoma in PSC patients are 7%, 8-11%, and 9-20%, respectively 6
- Older males with large-duct PSC and possibly concurrent ulcerative colitis are at an increased risk of developing cholangiocarcinoma 6