Cholangiocarcinoma

Cholangiocarcinoma is a type of primary liver tumour that originates in the bile ducts, also called cholangiocytes (the channels that transport bile from the liver to the intestine) and can develop inside (peripheral cholangiocarcinoma) or outside of the organ (extrahepatic cholangiocarcinoma). In more advanced stages, a malignant tumour such as cholangiocarcinoma has the ability to spread its cancer cells, through blood or by proximity, to other organs and tissues, causing the formation of secondary malignant neoplasms in new locations such as the lungs, brain, and bones.

Causes
Although the precise causes of the aforementioned genetic mutations at the origin of cell proliferation have not yet been identified, numerous scientific studies have concluded, with a certain degree of certainty, that they have identified several risk factors:

The most significant is the presence of primary sclerosing cholangitis, a serious chronic inflammatory disease of the liver, which first causes a stenosis or an abnormal and unnatural narrowing of the bile ducts that pass through the liver, and subsequently hepatic cirrhosis and hepatic failure.
Cairoli syndrome has been associated with the development of cholangiocarcinoma: it is a hereditary disorder affecting the bile ducts.
The presence of intrahepatic bile stones.
Exposure to certain chemicals and certain toxins, such as Thorotrast which, before the discovery of its carcinogenic power, was used as a contrast agent in various diagnostic X-ray procedures.
Parasitic infections (parasitosis) of the liver quite rare in the Western world, but present in countries such as Thailand, Taiwan, China, Eastern Russia, Korea, and Vietnam.
Hepatitis B and hepatitis C, both infectious diseases of the liver of viral origin.
Liver cirrhosis
Lynch II syndrome
Diabetes mellitus
Obesity
Excessive consumption of alcohol
Cigarette smoke.

Note that while it is a fairly rare tumour, in the presence of significant risk factors and when an individual falls in the demographic profile for cholangiocarcinoma, it is advisable to discuss it with your general practitioner, who will start an adequate diagnostic investigation when appropriate.

Symptoms
Cholangiocarcinoma accounts for 20% of primary liver tumours and develops primarily in males around 70 years of age.
The signs identifying the presence of the disease generally develop when the cholangiocarcinoma reaches certain dimensions and blocks the flow of bile along the biliary tract causing it go back towards the liver, from which it comes. Thus the substances contained in it then pass into the blood, causing symptoms such as:

weight loss
cachexia
pain in the upper part of the abdomen sometimes irradiated to the shoulder
jaundice
dark urine
yellowish diarrhoea
obstruction of the biliary tract due to the presence of bile salts in the urine
fever of 38°C (100°F) or higher.

The presence of jaundice is always a valid reason for contacting the primary physician in a timely manner or going directly to the nearest hospital for evaluations.
Do not overlook the other symptoms especially if protracted over days. An early diagnosis is in fact associated with a greater chance of recovery: for people with a cholangiocarcinoma found at a fairly early stage, the 5-year survival rate from diagnosis is between 20 and 50%.

Diagnosis
It is not always easy to diagnose cholangiocarcinoma, especially when the patient does not present the obvious signs such as jaundice, alteration of faeces, and dark urine that indicate the pathology.
Diagnostic investigations for the identification of cholangiocarcinoma begin with a careful study of the patient's history and a thorough physical examination, followed by blood tests, a series of diagnostic imaging tests, and tumour biopsy.
With a biopsy, in addition to obtaining a possible confirmation of the malignant nature of the tumour mass, it will be possible to establish the degree of malignancy and stage, that is, the infiltration power and metastasizing capacities of the cholangiocarcinoma.

Treatment
Treatment of cholangiocarcinoma depends on several factors including the stage of the disease. The treatment options are:

Surgical removal
Radiotherapy
Chemotherapy
Biliary stenting and biliary drainage by means of a bypass

These are almost always complex cases that require multi-disciplinary management to develop the most suitable strategy for the individual patient.