Ask the expert: CHOLANGIOCARCINOMA

“Cholan….a what?” I hear you say. Well, exactly. How often do you hear about that? So, as February is cholangiocarcinoma awareness month, I decided to raise some of that awareness by asking an expert all about it.

Allow me to introduce you to Professor Marco Novelli:

Prof Marco Novelli

Prof Novelli is a gastrointestinal histopathologist based at University College Hospital, London. He has specialised in gastrointestinal pathology for over 15 years with other specialist areas including inflammatory bowel disease, colon cancer and rare stromal tumours of the intestines.

It’s been a privilege to get the opportunity to ask him a collection of my own questions about cholangiocarcinoma, as well as those I gathered from my colleagues at the lab, too. And here they are:

What is cholangiocarcinoma?

“Cholangiocarcinoma is a malignant tumour which arises from the epithelium (lining cells) of the bile ducts – either within the liver (intra-hepatic) or in the large bile ducts draining into the duodenum (extra-hepatic).”

Anatomical location of the bile duct [image credit: wikicommons]

Are there any symptoms or warning signs?

“Cholangiocarcinomas typically present at a relatively late stage in their development. They may present with jaundice (due to blocking the flow of bile) but often symptoms are rather vague, such as abdominal pain and weight loss.”

Is there a particular demographic at higher risk of cholangiocarcinoma?

“In developed countries, primary sclerosing cholangitis (PSC) is the main risk factor for cholangiocarcinoma (although most cases occur in patients without PSC) and there is an increased risk in patients with developmental abnormalities of the hepatobiliary system. In developing countries of the far east, the main risk factor is chronic infection with liver flukes (for example Clonorchis sinensis).”

How common is it?

“It is relatively rare. There are approximately 1000 new cases in the UK every year.”

Have you ever reported a case of cholangiocarcinoma?

“Yes. We’ve seen cholangiocarcinoma in biopsies and resection specimens of the liver and pancreas”

How is it diagnosed?

“Typically it is picked up by a combination of abnormal blood tests (diagnosing jaundice) and imaging (CT scan, ultrasound scan, Magnetic Resonance Cholangiopancreatography, or MRCP, etc). An Endoscopic Retrograde Cholangiopancreatography, or ERCP, may be performed where radiocontrast is injected into the bile ducts from the duodenum (via the Ampulla of Vater) to outline the biliary tract and show any abnormalities.”

Do you ever need to use immunohistochemistry to aid/confirm diagnosis?

“On biopsy material we often need to use immunohistochemistry to exclude metastatic tumour from other sites in the body such as the colon. The immunophenotype of cholangiocarcinoma is pretty non-specific: CK7 positive with or without CK20 positivity, but usually CDX2 negative. Diagnosis is often based on a combination of radiological findings (including lack of another primary site) and the immunoprofile.”

How aggressive can it be?

“It tends to be a very aggressive tumour which presents at a relatively late stage.”

Can it metastasise?

“It often metastasises both within and to the liver and to local lymph nodes. Widespread dissemination (carcinomatosis) can also occur.”

Is it related to or associated with any other conditions?

“Yes, it is often associated with primary sclerosing cholangitis (an autoimmune disease of the bile ducts which is associated with ulcerative colitis) and congenital hepatobiliary abnormalities.”

What are the risk factors?

“The main risk factor is age as most cases occur in the over 65s, but PSC and hepatobiliary malformations also act as risk factors, as already discussed.”

Can it be prevented?

“Eradication of liver fluke infestation in the population of developing countries would reduce the global incidence, but otherwise I am unaware of any preventative measures.”

How can it be treated?

“If caught early enough it can be treated by surgery, which offers the only chance of a cure – typically partial hepatectomy for intra-hepatic tumours or pancreatectomy for extra-hepatic tumours. In most cases treatment is palliative with chemotherapy, radiotherapy, debulking of tumor (for example: laser therapy or photodynamic therapy) or tumour stenting.”

What are the survival rates?

“The survival rates are very low.  Most cases (approximately 80%) are inoperable at presentation, and these patients rarely survive 5 years from diagnosis. Of the 20-25% who are operated on, less than half survive 5 years.  Extra-hepatic tumours do slightly better than intra-hepatic tumours.”

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As this is an awareness month, Prof Novelli has also generously offered to continue to take any further questions you may have in addition to those answered above. So, please do make the most of this great opportunity to ask him your questions about this month’s hot topic. You can submit your question(s) a number of ways:

•  post in the comments box below

• @reply on twitter to me at (@dellybean) or (@HistoQuarterly)

•  or email me using the contact button on my homepage.

We look forward to hearing from you.

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Useful links

The Cholangiocarcinoma Foundation.

The Alan Morement Memorial Fund Cholangiocarcinoma Fund.

What you can do to help:  this year, the AMMF are focusing Cholangiocarcinoma Awareness Month on GP awareness. As part of this year’s campaign, they are encouraging people to hand in this letter [pdf] to GPs in an attempt to increase the number of people being diagnosed sooner, making them eligible for life saving surgery.