What are cysts and tumours?
There are various types of cysts and tumours in the liver, all of which are abnormal growths or structures.
A cyst is an abnormal, fluid-filled balloon or bubblelike structure (sac) that can grow in any part of your body. Liver cysts are mostly congenital, meaning that you have them from birth, or caused by contact with some form of parasite.
A solid tumour is an abnormal lump or swelling of tissue caused by an uncontrolled build-up of cells. Tumours can be benign or malignant – those that are benign do not spread or cause cancer, while malignant tumours are cancerous and may invadeother parts of the body.
Most of the information is focused on cysts and how they can affect the liver and bile ducts. Bile ducts are tube-shaped structures that carry bilefrom the liver to the gallbladder and then to the small intestine for removal (excretion) from the body.
Benign liver tumours
Benign liver tumours are common and usually without symptoms (asymptomatic). They tend to be found during imaging tests for unrelated conditions and, although most need no treatment, it is important for doctors to tell these tumours apart from those that are malignant.
Haemangiomas are the most common of all benign solid tumours. Around 1% of people may have one of these tumours. Haemangiomas are made up of newly formed but abnormal blood vessels. They can vary in size but are usually less than 5cm (or a couple of inches) and usually remain so once grown.
Haemangiomas are usually picked up on ultrasound scanning. Further imaging techniques such as computed tomography (CT) and magnetic resonance imaging (MRI) or microbubble ultrasound may be required to confi rm the diagnosis. A CT scan examines the density (thickness) of your body tissues. It obtains pictures, called tomograms, from different angles around your body using computer processing and can also show very detailed cross-sectioned images of your tissue and organs. With MRI a special tube scanner is used to provide a detailed view of your internal organs.
The majority of haemangiomas do not require long term monitoring or treatment. In rare cases where a tumour becomes overly large, embolisation (a type of treatment aimed at reducing the blood supply to the tumour) and surgery are most commonly used to reduce or remove it.
Focal nodule hyperplasia (FNH)
After haemangiomas, FNH is the most frequent of the benign solid liver tumours. It is commonly found in women aged between 15 and 50. The typical tumour is a small mass or lump commonly measuring between 3cm to 5cm in diameter and is usually found singly. It is characterised by a pale-coloured central scar that in most cases can be seen in CT and MRI scans.
Some people experience pain in their abdomen (the ‘tummy’ or ‘belly’ area between the chest and pelvis) but most do not have clear symptoms. These tumours do not rupture or become malignant and do not need specific treatment.
Adenomas are tumours that can affect a number of organs and originate in your glands. Hepaticadenomas are very rare and are thought to be linked to exposure to certain hormones. On average, these tumours can measure between 8cm to 15cm. Of the benign tumours, they require the most clinical attention because it is possible for them to grow (and press against other organs or tissue), rupture and bleed. If this does happen it can cause severe abdominal pain.
The risk of rupture is increased in pregnancy, so that increased monitoring by ultrasound is advisable. Ultrasound scans involve sending sound waves into the body to create echoes that can be recorded and used to build a picture of the liver’s condition.
In the extremely rare case that an adenoma does become malignant, surgical removal (resection) maybe advised.
The overwhelming majority of cystic disease is caused by simple cysts. There are also inherited disorders that cause cysts, including polycystic liver disease, congenital hepatic fibrosis and Caroli’s syndrome. Although rare in the UK, hydatid cysts are caused by a parasitic infection.
The bile ducts in the liver are like the branches of a tree that come together just below the stomach.This is known as the biliary tree, sometimes called the biliary system or biliary tract. A side branch ofthe biliary tree leads to the gallbladder, the organ that stores bile. Gallbladder disease involving the biliary tree is more common than cystic disease.
Cysts affecting the biliary tree belong to a group of diseases caused by congenital bile duct abnormality and are usually inherited. These cysts can create problems by:
placing pressure on surrounding organs and body tissue as they become enlarged
causing inflammation of the bile ducts, known as cholangitis
- causing increased blood pressure in the portal vein, known as portal hypertension.
Simple liver cysts
Simple cysts arise from a malformation of your bile ducts. In rare cases, they can cause bile duct obstruction, secondary infection or may rupture. Some may require surgical drainage.
Simple cysts are not inherited and unrelated to polycystic liver disease in which many more cysts are present. They are estimated to affect between 1% and 2.5% of the population. They affect all ages, but occur more frequently in the population as age increases.
Simple cysts usually have no symptoms. Larger cysts may occasionally cause some dull pain and/ or swelling in the upper right hand side of your abdomen, an area referred to as the ‘right upper quadrant’. Jaundice (yellowing of the skin and whites of the eyes), which is often caused by bile duct obstruction, is rare.
To diagnose simple liver cysts medical staff may record your medical history and carry out a physical examination. An ultrasound or CT scan of your abdomen may also be carried out. Simple liver cysts generally do not require treatment unless they become large enough to cause pain.
If required, to reduce their size, doctors can remove the fluid from the cyst. They do this by inserting a needle through the skin under X-ray guidance, and withdrawing (aspirating) the fluid. However, the fluid will usually refill the cyst. To help stop this, after the fluid has been taken out, doctors may inject a substance into the cyst to help stick the walls together. This substance is called a sclerosant. A similar technique involves removing part of the wall of the cyst, called laparoscopic de-roofing.
Polycystic liver disease (PLD)
Adult polycystic liver disease is an inherited disorder where many cysts of various sizes develop throughout your liver and is associated with congenital hepatic fibrosis. Both conditions have in common a growth of numerous abnormally shaped (malformed) bile ducts, known as ductal plate malformation. PLD may occur with polycystic kidney disease (PKD) or alone but is less common and milder than the kidney form.
The tendency to form multiple cysts is probably present at birth, but cysts usually do not become larger and cause problems until you are an adult.
Cysts can be very small, perhaps no larger than a pinhead, but can grow up to 10cm. Similarly, your liver can remain its normal size or become heavily enlarged. Your liver should continue to function effectively in PLD and the disease is not considered to shorten your life expectancy. PLD becomes more common with age but is rare, affecting much less than 1% of the population.
Women tend to be more affected as the size and increase of cysts is thought to be linked to levels of oestrogen.
Polycystic liver disease is first noticed during puberty, with symptoms becoming more noticeable as you enter your thirties. Most people are diagnosed in their forties and fi fties. PLD is often without clear symptoms, but swollen, painful abdomen and an enlarged and hardened liver (hepatomegaly) are usually associated with the condition. In many cases polycystic liver disease is discovered by accident or noticed during diagnosis of kidney disease. The majority of people with polycystic kidney disease will also have PLD.
Laboratory tests such as liver and renal function tests may be used to diagnose PLD. Liver function tests (LFTs) are used to indicate whether your liver is inflamed (hepatitis), damaged or not working properly. They measure levels of certain enzyme and protein substances in your blood that may alter when liver damage is present. Doctors will also use imaging tests such as ultrasound, CT or MRI scan and a special X-ray of the kidney (intravenous pyelogram).
Cysts rarely require treatment. If necessary, laparoscopic de-roofing can be helpful for reducing larger, uncomfortable cysts. In rare cases of severe PLD, where multiple cysts cause the liver to become massively enlarged and very painful (leading to other complications), a liver transplant may be required.This is usually only recommended if your quality of life has become very poor and other treatments are no longer helpful.
Congenital hepatic fibrosis (CHF)
Congenital hepatic fibrosis is an inherited disease that affects both your liver and your kidneys. CHF causes scarring and hardening of the liver, which makes it more difficult for the blood to flow through it. This causes a condition called portal hypertension, where there is increased pressure in the veins that carry blood to the liver. Bleeding oesophageal varices – small, protruding veins that line your gullet(oesophagus) – are an early sign of this condition.
If you have CHF your kidney function may also be affected. Some scientists believe that CHF and another condition called autosomal recessive polycystic kidney disease (ARPKD) are part of the same disorder.
Both CHF and ARPKD are very rare, affecting around one person in 20,000 and symptoms occur more in newborns and young children.Symptoms of CHF may be apparent from early childhood. These may include abdominal pain. Your kidneys and spleen may also be enlarged. Complications from portal hypertension may cause bleeding in the stomach, vomiting blood and blood in your faeces (from oesophageal varices). This condition is usually diagnosed in childhood, either because you have an enlarged liver or because of bleeding varices. In some people the diagnosis is not made until adult life. In addition to liver function tests, diagnosis is made by carrying out:
an ultrasound scan of your liver and kidneys
a CT scan of your abdomen
an X-ray of blood vessels (angiography)
an endoscopy to detect varices
a liver biopsy.
CHF differs from cirrhosis because your liver cells remain able to function. There is no specific treatment for the condition but many people require re-routing of blood from the intestines. This is known as a ‘shunt’ operation and is carried out to prevent more intestinal bleeding. A metal or plastic tube (stent) is used to join two veins in order to change the direction of blood fl ow. It is usually done by a radiologist with the help of ultrasound or other imaging equipment to guide them. This procedure will mean at least a day or two in hospital.
Bleeding oesophageal varices can be treated during endoscopy. This is a procedure in which a long,flexible fibre optic tube with a tiny camera on the end (endoscope) is passed into your gullet after you have been sedated.
The bleeding is often treated using a technique called banding, often carried out as day surgery. Banding is where a vein is sucked into a small chamber attached at the end of the endoscope and a small band is placed around its base (ligation).
Complications from bleeding varices are life threatening and early diagnosis is important. When these procedures are effective, without other complications, the prognosis for people with CHF is usually good.
There are different types of choledochal cysts. They are categorised by their size and where they are found. They affect the main trunk of the biliary tree, known as the common bile duct, causing it to become unusually enlarged. This is called cystic dilatation and probably occurs at time of birth. It leads to an abnormal junction between the common bile duct and the pancreatic duct (anomalouspancreatobiliary junction, or APBJ).
Usually by the age of two or three, but sometimes not until you have reached adulthood, the duct may form a cyst. This prevents the bile from reaching the intestine. Bile backs up into the liver, causing you to become jaundiced. Inflammation and weakening of the bile duct walls will follow.
In childhood this may lead to pancreatitis (inflammation of the pancreas), cholangitis and liver damage. The most worrying complication may be cholangiocarcinoma, a rare type of cancer that develops in cells lining the bile ducts in the liver.
Choledochal cysts are extremely rare. The condition affects females more than males and occurs more commonly in Southeast Asia, with more than 30% of reported cases occurring in Japan. The majority of choledochal cysts are seen in childhood, although some people may not show symptoms until they are adults. Symptoms are jaundice, caused by the reduced amount of bile draining from the abnormal bile duct,and abdominal pains. Pain may be increased if the bile building up in the liver becomes infected. In some cases the cyst can be felt by the doctor when examining your abdomen.
The diagnosis of choledochal cysts is not difficult once the condition is suspected. Cysts can be confirmed by using imaging equipment such as ultrasound, MRI or magnetic resonance cholangiopancreatography (MRCP), endoscopic retrograde cholangio-pancreatography (ERCP) and CT.
During an ERCP, the endoscope is passed into the duodenum and contrast medium is injected back up into the bile ducts. This can be visualised with X-ray screening allowing the gastroenterologist to see the outline of your biliary tree.ERCP is an endoscopic procedure which, although invasive, is valuable in examining both your biliary and your pancreatic structures. It can identify problems with the biliary tree, cystic duct obstruction, gallstones, narrowings and tumours.
If the condition is not correctly diagnosed the blockage of bile may result in scarring (fi brosis) of your liver. Extensive scarring may lead to cirrhosis. If you are diagnosed as an adult, inflammation and damage to the bile ducts may have been occurring for many years.
Choledochal cysts are treated by surgical removal of the abnormal segment of the bile duct and reconstruction of the biliary tree so that bile travels back into the intestine.
Caroli’s disease and Caroli’s syndrome are biliary tree disorders in which small cysts alternate with narrowed segments of the bile ducts. These abnormalities may be present throughout the liver, or limited to a small area. They are more common in adults and more likely to occur in women.
Caroli’s disease, which occurs in the main bile ducts, is less common than Caroli’s syndrome although both are very rare. In Caroli’s syndrome the small bile ducts are also malformed and congenital hepatic fibrosis occurs.
Caroli’s syndrome is generally inherited, whereas Caroli’s disease is not. As with congenital hepatic fibrosis, Caroli’s syndrome is often associated with autosomal recessive polycystic kidney disease (ARPKD).
People with Caroli’s disease and Caroli’s syndrome may have cholangitis, portal hypertension and, as with people who have choledochal cysts, are at much higher risk of developing cholangiocarcinoma.
The symptoms are usually abdominal pain and, very rarely, jaundice. Complications from portalhypertension may cause vomiting of blood (haematemesis) and blood in your faeces (maelena).Bleeding comes from burst or ruptured varices. If the bile becomes infected, you may develop fever, abdominal pain and, rarely, jaundice. This complication can first appear in childhood or may not occur until middle age.
Doctors will be looking for bile duct abnormalities and evidence of fibrosis. LFTs, imaging and liver biopsy may all be used in diagnosis. Radiologists will scan the bile ducts using ultrasound, CT and MRCP. ERCP and another invasive technique called percutaneous transhepatic cholangiography (PTC) may also be used. With PTC, a thin needle is passed through your skin and through the liver into a bile duct. A dye is injected so that the biliary tree becomes outlined on X-ray. This picture will show any narrowing or blockages. If severe cholangitis is suspected, a liver biopsy may also be carried out.
Treatment may be required if you have recurrent or severe cholangitis. This can involve reconstructive surgery to improve the flow of bile. If you have developed gallstones, these may be removed by an ERCP procedure.A medication made from naturally occurring bile acids called ursodeoxycholic acid (URSO) may also be given to soothe any complications arising from gallstones.
This type of disease is a parasitic infestation by a tapeworm known as ‘echinococcus granulosus’. This parasite, which lives mainly in dogs, enters your body as larvae. This is likely to happen when you handle an infested animal or eat food or drink water contaminated by its faeces. The larvae travel in the bloodstream and lodge in organs to form cysts. The liver is most commonly affected (70% of hydatid cysts form there), followed by the lungs, brain and bones.
The cysts become larger and in some cases form a mass. This can take decades, and not all cysts are active. Growing cysts can cause damage to your body tissues either directly or by affecting the flow of blood. They can also rupture or leak, possibly leading to allergic reaction and shock (anaphylaxis). If the rupture affects biliary ducts, cholangitis may follow. More seriously, secondary infection can occur if this happens in your lungs. A ruptured cyst will also cause the disease to spread further in your body.
Hydatid cysts are very rare in the UK and are more likely to be contracted abroad where there has been contact with sheep dogs and other animals. These areas are more commonly southern Europe, the Middle East, Australia, New Zealand and South America.
A related parasite, echinococcus multilocularis, is found in the northern hemisphere. It lives in wild foxes and is passed on through contact with rodents. It enters the human body in the same way as echinococcus granulocus but the cyst behaves more like a cancerous tumour.
Both parasites can cause severe liver disease but are very rare. They are estimated to affect only one person in 100,000 in the UK. Symptoms may be at first general and can be non-specific. They are generally caused by pressure from the size of the cysts. Possible symptoms may include:
abdominal pain and/or swelling and tenderness
Because the parasite grows slowly in your body it is rarely diagnosed during childhood or adolescence unless your brain is affected. Diagnosis of hydatid disease is usually reached by blood tests and imaging tests.
Liver function tests are used, together with a test mainly in immunology to detect the presence of antigens or antibodies, known as the enzyme-linked immunosorbent assay (ELISA). Antigens are invading or foreign proteins, and antibodies are the proteins your body produces to defend against them.
CT and ultrasound scans are most commonly used to show single or multiple cysts. MRI scans may be used to provide greater detail to help doctors decide whether cysts are active, inactive or something in between (transitional). ERCP may be used to show cysts in the bile ducts.
Drugs are usually used first in the treatment of hydatid disease to help prevent any spread of the disease although surgery is still required in many cases. The fi rst line drug treatments are albendazole and mebendazole. These drugs are given in oral form and work by being absorbed through the cyst’s membrane to affect its growth. You will have to take these drugs for three months. Another drug, praziquantel, can be given over 14 days and is now sometimes used in combination with albendazole and mebendazole.
Between a third and half of cysts treated with drugs may grow smaller or disappear. If surgery is required, the aim is usually to remove the cyst completely. This can be complicated, particularly where there has been secondary infection from the cyst, and in certain cases may carry significant risk of mortality.
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Reviewed by:Dr Ashley Brown, Consultant Hepatologist, Imperial College Healthcare NHS Trust
The Trust has been donated the use of this video discussing the physiology of Benign Tumours – it is aimed at medical students but after asking for feedback, some patients have also said they find it useful. It can be quite technical, so please discuss any content that you are unsure about with your medical team.