Advanced/Metastatic Cholangiocarcinoma Treatment Landscape - Episode 1

Identification and Diagnosis of Cholangiocarcinoma

Anthony El-Khoueiry, MD, reviews cholangiocarcinoma subtypes and outlines strategies in identification and diagnosis.


Anthony B. El-Khoueiry, MD: Cholangiocarcinoma continues to be a relatively rare tumor. But a subset of it, what it’s known as intrahepatic cholangiocarcinoma, seems to be on the rise over the past couple of decades. It continues to increase in incidence for reasons that are not completely specified. In general, when we talk about biliary cancers, we’re talking about a few sites of disease. We might be talking about intrahepatic cholangiocarcinoma. We might be talking about hilar cholangiocarcinoma, previously known as Klatskin tumors. We might be talking about extrahepatic cholangiocarcinoma, which usually involves the common bile duct, but not ampullary cancer. We might be talking about gall bladder carcinoma. There are different subsets within the umbrella of biliary cancers that’s important to keep in mind.

As far as diagnosis, there are 2 issues to keep in mind. One is that extrahepatic and Klatskin tumors can be difficult to diagnose because of the difficulty in obtaining tissue verification. Often times, if this is a localized tumor, it requires an ERCP [endoscopic retrograde cholangiopancreatography] with brushings. The yield can be low and may have to be repeated, and sometimes the assistance of an endoscopic ultrasound may increase the yield. That’s 1 diagnostic challenge that can be an issue. If the disease is resectable—and if it definitely looks like cancer, even without being able to verify the diagnosis of cancer on tissue—sometimes the surgeons may proceed to operate.

As far as intrahepatic cholangiocarcinoma, these tumors historically were frequently reported as cancers of unknown origin, or unknown primary cancers, but we’ve learned over time how to diagnose intrahepatic cholangiocarcinoma. Part of it is a diagnosis of exclusion. Typically these patients present with liver tumors—it could be 1 or multiple—and end up having a biopsy, and the way to diagnose intrahepatic cholangiocarcinoma 1 is the histology has to be consistent. It has to be an adenocarcinoma. It has to have the right histologic appearance. Usually, these are CK7-positive, CK20-negative tumors. They may be CA19 [cancer antigen 19]–positive as well. And there’s a diagnosis of exclusion. We make sure that there’s no mass in the stomach, in the esophagus, and that there’s no other upper–GI [gastrointestinal] primary and there’s no other primary elsewhere. Doing an endoscopy or colonoscopy isn’t mandated. If the clinical presentation and the histology are consistent with intrahepatic cholangiocarcinoma, it would be sufficient to call it that.

The other thing I would add is that these patients can have delayed diagnoses, especially the intrahepatic cholangiocarcinoma, because the liver is a large organ, the tumors can grow silently, and patients can present with malnutrition, pain, and significant failure to thrive. Paying attention to nutritional status and performance status, doing interventions to improve nutrition, and the activity level of the patient with physical therapy are important because performance status is a definite prognostic indicator for the outcome of patients with these types of cancers.

Transcript edited for clarity.