Brief overview
- Symptoms: Among others, stool discoloration, dark urine, itching (pruritus), weight loss, pain in the upper abdomen, nausea, vomiting.
- Causes and risk factors: The cause is not precisely known. The most important risk factor is age; in addition, certain diseases favor bile duct cancer (for example, bile duct stones or parasitic diseases).
- Diagnosis: Physical examination, liver values (blood test), various imaging procedures such as ultrasound examination.
- Course and prognosis: Since the tumor is usually discovered late, when the disease is already well advanced, the prognosis is rather unfavorable.
What is cholangiocellular carcinoma?
A cholangiocellular carcinoma (CCC, cholangiocarcinoma, bile duct carcinoma) is a malignant (malignant) tumor of the bile ducts. The cancer is one of the primary liver tumors, as is hepatocellular carcinoma (HCC).
Anatomy of the bile ducts
The liver produces 600 to 800 milliliters of bile (bile) daily. This enters the intestine through the bile ducts. The bile ducts begin as the smallest bile capillaries between the liver cells and then merge to form larger bile ducts. They combine to form a right and left hepatic duct.
This gives rise to the common hepatic duct (ductus hepaticus communis). From it, a duct branches off to the gallbladder (ductus cysticus). It then runs as the ductus choledochus to the duodenum, where it joins the pancreatic duct (ductus pancreaticus).
Types of cholangiocellular carcinoma
Physicians divide cholangiocelular carcinoma into three types according to its anatomical location:
- Intrahepatic CCC (located in the liver; extending to the right and left hepatic ducts).
- Perihilar CCC (so-called Klatskin tumor; located as far as the choledochal duct)
- Distal CCC (extending to the duodenum)
Symptoms
Cholangiocellular carcinoma often causes no symptoms for a long time. Therefore, the diagnosis of the bile duct tumor often occurs only at an advanced stage. Symptoms that sufferers with bile duct carcinoma have include:
- Stool discoloration
- Dark urine
- Itching (pruritus)
- Weight loss
- Pain in the upper abdomen
- Loss of appetite
- Nausea, vomiting
- Fluid accumulation in the abdomen (ascites)
Cholangiocellular carcinoma: Causes and risk factors.
The exact cause of cholangiocellular carcinoma is unknown. Often, apart from an older age, no special risk factors can be found in the affected persons. Only some diseases are known to favor the development of bile duct cancer. These include:
- Enlargements of the bile ducts outside the liver (choledochal cysts)
- Bile duct stones (choledocholithiasis)
- Parasitic diseases of the bile ducts (for example trematodes or liver fluke)
- Primary sclerosing cholangitis (also PSC, an inflammatory disease of the bile ducts)
Possible additional risk factors may include hepatitis B and C infections, cirrhosis of the liver, alcohol and nicotine use, and diabetes mellitus.
Examinations and diagnosis
- Whether there has been any unwanted weight loss recently
- Whether the skin is itchy
- Whether the stools are lighter or the urine darker than usual
- Whether the affected person vomits more frequently
Physical examination
Laboratory tests
In addition, the physician draws blood from the affected person if cholangiocellular carcinoma is suspected. He has it examined in the laboratory for certain values that are frequently altered in bile duct carcinoma. These include the liver enzymes alanine aminotransferase (ALAT), aspartate aminotransferase (ASAT), glutamate dehydrogenase (GLDH), gamma-glutamyltransferase (γ-GT) and alkaline phosphatase (AP). They are all frequently elevated in liver damage.
Further diagnostics
If the physical examination and laboratory results provide evidence of cholangiocarcinoma, the physician may perform an ultrasound examination (sonography) of the abdomen. It also happens that the doctor accidentally detects a cholangiocellular carcinoma during a routine ultrasound examination.
For further clarification, physicians usually perform a magnetic resonance imaging (MRI) or computed tomography (CT) scan to detect cholangiocellular carcinoma.
He then takes an X-ray of the abdomen, on which the contrast medium can then be seen. It should be distributed in the bile ducts. If it leaves out a bile duct, for example, this is an indication of a stone or a tumor.
An alternative to ERC is percutaneous transhepatic cholangiography (PTC). In this procedure, the physician also injects contrast medium into the bile ducts, but in this case via a needle that he advances through the skin and liver into the bile ducts under X-ray control.
It is also possible to diagnose cholangiocellular carcinoma with the help of magnetic resonance imaging (MRI) or computed tomography (CT).
Cholangiocellular carcinoma: treatment
If surgery is not possible or not successful, there are palliative treatment options. This is the case, for example, if metastases of the tumor have already formed in other organs. Palliative means that a cure is no longer possible, but that the patient’s symptoms can be improved by the therapy.
In addition, the doctor often inserts a stent into the bile ducts as an adjuvant therapy. This is a small tube that keeps the bile ducts open so that the bile can drain more easily. In some cases, the doctor may also try to keep the bile ducts open with the help of radiofrequency or laser therapy.
Disease progression and prognosis
Cholangiocellular carcinoma usually has a poor chance of cure. This is mainly due to the fact that in many cases it causes symptoms at a late stage and is therefore only detected at a late stage.
