PatientPlus articles are written for doctors and so the language can be technical. However, some people find that they add depth to the articles found in the other sections of this website which are written for non-medical people.

Liver tumours and cysts

Tumours of the liver may be benign or malignant and the latter may be primary or secondary. The commonest cause of liver tumours is metastatic carcinoma.
The vulnerability of the liver may be related to the fact that it is the largest organ in the body and it filters blood from both the systemic and portal systems. Humoral factors promote cell growth and blood flow is second only to the lungs.

The more common causes of liver tumours are listed below:

Cystic disease of the liver:

• Infantile polycystic kidney disease is inherited as an autosomal recessive. There are 4 categories of the disease called perinatal, neonatal, infantile and juvenile. In general, the later the onset of the condition, the less the kidneys are affected by cysts and the more the liver is involved so that in the juvenile form there is variable renal involvement, marked hepatic cysts, portal hypertension and death is more often from liver failure than chronic renal failure. Mortality is the lowest of the 4 categories, with around 80% surviving beyond the age of 15 years. In adult polycystic kidney disease the liver is not involved.
• Polycystic liver disease does exist as a separate entity from polycystic kidney disease but is rarer. It is listed by OMIM as an autosomal dominant.¹ The natural history is rather variable with pain as the usual presentation. Some develop portal hypertension and a few need liver resection and even transplantation.²
• Simple hepatic cysts are of unknown aetiology. They are thought to be congenital in origin. They are solitary and lined by biliary epithelium. If they grow to great size they cause dull right upper quadrant pain. They may produce hepatomegaly with a liver palpable below the costal margin. Diagnosis is by ultrasound and the best treatment is laparoscopic fenestration of large simple cysts.³
• Parasitic cysts are usually caused by the tapeworm Echinococcus granulosus and called Hydatid cysts or they may be the result of amoebiosis. Small hydatid cysts are often asymptomatic but larger ones may cause pain and rupture, and this may produce anaphylactic shock. Medical treatment is usually ineffective and so surgical removal is required but with great care not to spill the contents.⁴
  Amoebic abscess usually causes pyrexia, pain and weight loss. Fluid aspirated under imaging guidance is said to be like anchovy sauce. It usually responds to amoebocides.
• Liver abscess is usually pyogenic rather than parasitic in the UK. In neonates it is often related to umbilical vein catheterisation but in children and adolescents there is usually trauma or immune compromise. In older people it is often associated with malignancy. The right lobe is affected twice as often as the left with just 5% being bilateral.

Benign tumours can often be diagnosed by imaging and immunohistochemistry without recourse to biopsy.⁵

• Haemangioma is the commonest benign tumour of the liver. It is usually asymptomatic and may be found on ultrasound examination, perhaps for gallstones. They usually cause no problem unless an injudicious attempt is made at liver biopsy as the lesion may bleed profusely. They are thought to be congenital but may enlarge under the influence of oral contraceptives but usually regress when they are stopped. If they require removal this is not usually amenable to simple excision of the tumour and a whole lobe of the liver must be removed. A haemangioma is usually only 1 or 2cm in diameter but a cavernous haemangioma may reach 8 to 10cm in diameter. Haemangiomas grow during pregnancy and administration of oestrogens but a case control study failed to find a correlation between oral contraceptive use and haemangioma⁶
• Hepatocellular adenoma is also usually asymptomatic and found fortuitously. Hepatocellular adenoma is a rare condition but may be more common now because of oral contraceptives.⁷ Hepatic adenoma and adenocarcinoma are also associated with use of anabolic steroids.⁸ This may be abuse by body builders or legitimate medical use. There is about a 5% chance of malignant change and so they are best removed.

Focal nodular hyperplasia is a benign proliferation of hepatocytes occurring around an abnormal artery in the liver. It is generally smaller than 5cm in diameter and in the centre of the nodule is the abnormal artery. No treatment is required as it is asymptomatic, and is not prone to rupture or malignant change. This condition is often associated with haemangioma of the liver and oral contraceptives may facilitate the recognition by encouraging growth.⁹

Primary carcinoma of liver:

• Hepatocellular carcinoma accounts for 90% of primary liver cancers. In 80 to 90% of cases it occurs on a background of cirrhosis. In western societies alcohol abuse is the commonest cause of the cirrhosis but worldwide it represents the commonest cause of death from malignancy with an incidence in parallel with hepatitis B. Prevention of hepatitis B may represent a very important and valid way to immunise against cancer.
  Alpha feto-protein is elevated in 75% of cases and the higher the level the worse the prognosis.
• Hepatoblastoma is the commonest liver cancer in children, although it is rarer than other solid tumours in childhood. Surgical techniques and adjuvant chemotherapy have markedly improved the prognosis. Complete surgical resection of the tumour at diagnosis, followed by adjuvant chemotherapy, is associated with 100% survival rates, but the outlook is bleak for children with residual disease after initial resection, even if they receive aggressive adjuvant therapy. Hepatoblastoma is associated with prematurity and low birth weight.¹⁰ It is also associated with Gardener's syndrome or familial polyposis coli.¹¹
• Cholangiocarcinoma arises in the biliary tree anywhere from the small intrahepatic ducts to the distal common bile duct. It is most commonly found at the junction of the left and right hepatic ducts. It presents by causing obstruction to the flow of bile. The commonest cause of malignant obstruction of the bile ducts is carcinoma of pancreas. Gall bladder cancer is 9 times more common than bile duct malignancy.

Metastatic malignancy of the liver: Lymph nodes are the commonest site for metastatic malignancy with the liver next in frequency. Most liver metastases are multiple with 77% affecting both lobes. Single metastases occur in only 10%.

The commonest primary sites of hepatic metastases are colo-rectal carcinoma, carcinoma of stomach, carcinoma of pancreas, breast cancer and lung cancer. In children the commonest primaries are neuroblastoma, Wilm's tumour and leukaemia.

Around half of patients with metastatic liver disease have hepatomegaly and ascites. Abnormal LFTs are non-specific and unreliable. Most tumours that have spread to the liver have metastasised to other sites too.

The extent of metastatic liver disease, as shown on imaging, may be surprising considering how limited are physical symptoms and signs. There may be nodularity of the palpable edge of the liver but 30% of patients have a normal size liver and 10% have no surface involvement. Large metastases may block bile ducts and cause jaundice, malaise, anorexia and loss of weight. Ascites indicates wide dissemination and a poor prognosis. The spleen is often enlarged without portal hypertension. Lower limb oedema suggests blockage of the inferior vena cava. Carcinoid tumours can cause pulmonary stenosis and this almost invariably indicates hepatic involvement.

References:

1. OMIM Polycystic liver disease
2. Bistritz L, Tamboli C, Bigam D, et al; Polycystic liver disease: experience at a teaching hospital.;Am J Gastroenterol. 2005 Oct;100(10):2212-7.[abstract]
3. Pitale A, Bohra AK, Diamond T; Management of symptomatic liver cysts.;Ulster Med J. 2002 Nov;71(2):106-10.[abstract]
4. Buttenschoen K, Carli Buttenschoen D; Echinococcus granulosus infection: the challenge of surgical treatment.;Langenbecks Arch Surg. 2003 Sep;388(4):218-30. Epub 2003 Jul 4.[abstract]
5. Choi BY, Nguyen MH; The diagnosis and management of benign hepatic tumors.;J Clin Gastroenterol. 2005 May-Jun;39(5):401-12.[abstract]
6. Gemer O, Moscovici O, Ben-Horin CL, et al; Oral contraceptives and liver hemangioma: a case-control study.;Acta Obstet Gynecol Scand. 2004 Dec;83(12):1199-201.[abstract]
7. Glinkova V, Shevah O, Boaz M, et al; Hepatic haemangiomas: possible association with female sex hormones.;Gut. 2004 Sep;53(9):1352-5.[abstract]
8. Socas L, Zumbado M, Perez-Luzardo O, et al; Hepatocellular adenomas associated with anabolic androgenic steroid abuse in bodybuilders: a report of two cases and a review of the literature.;Br J Sports Med. 2005 May;39(5):e27.[abstract]
9. Mathieu D, Zafrani ES, Anglade MC, et al; Association of focal nodular hyperplasia and hepatic hemangioma.;Gastroenterology. 1989 Jul;97(1):154-7.[abstract]
10. Feusner J, Plaschkes J; Hepatoblastoma and low birth weight: a trend or chance observation?;Med Pediatr Oncol. 2002 Nov;39(5):508-9.[abstract]
11. Gruner BA, DeNapoli TS, Andrews W, et al; Hepatocellular carcinoma in children associated with Gardner syndrome or familial adenomatous polyposis.;J Pediatr Hematol Oncol. 1998 May-Jun;20(3):274-8.[abstract]

Internet:

• Mulvihill SJ hepatic cysts; eMedicine; August 2002
• Willert JR hepatoblastoma; eMedicine; December 2003
• Khan AN liver, metastases; eMedicine; November 2003

Acknowledgements EMIS is grateful to the Mentor authoring team for writing this article. The final copy has passed peer review of the independent Mentor GP authoring team. ©EMIS 2006.

Last issued 05 Jul 2006