Hepatic cysts
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Editor-in-Chief: Elliot B. Tapper, MD. Department of Medicine. Beth Israel Deaconess Medical Center
Associate Editors-in-Chief: C. Michael Gibson, M.S., M.D., Cafer Zorkun, M.D.
Overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Liver cysts are common, detected in as much as 4-7% of the general population,[1][2] and are the result of many causes. Most cysts are found incidentally, on imaging studies ordered for other reasons altogether. Some cysts, however, can become symptomatic – causing pain, nausea, jaundice, compressing other abdominal structures, rupturing or compromising the function of the liver.[3] When symptomatic, an intervention is often indicated. As the causes are so diverse, it will be useful to discuss these cysts, their diagnosis and their management, separately.
References
- ↑ Caremani M, Vincenti A, Benci A, Sassoli S, Tacconi D. Echographicepidemiology of non-parasitic hepatic cysts. J Clin Ultrasound 1996;21:1 15-1 18.
- ↑ Sanfelippo PM, Beahrs OH,Weiland LH. Cystic disease of the liver. Ann Surg 1974;179:922–925.
- ↑ Sanfelippo PM, Beahrs OH,Weiland LH. Cystic disease of the liver. Ann Surg 1974;179:922–925
Historical Perspective
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References
Classification
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Classification
Bile Duct Hamartomas
Bile duct hamartomas (BDH) – also referred to as ‘simple cysts’ and ‘von-meyenberg complexes’ – are uncommon but not rare. They are benign and do not transform into malignancies. Small cysts can be found in as many as 7.3% of people referred for abdominal MRI while giant, symptomatic cysts are present in 0.4% of the population referred for such imaging.[1] BDH are more common in women, in a ratio of 4:1 or more, and the average age of presentation is 60 years old. [2][3] Pain is, by far, the most common presentation, but patients have also complained of jaundice, edema and have presented, rarely, with a ruptured cyst.
Cystadenoma and Cystadenocarcinomas
Less common than BDH, the largest published series describing these lesions found 18 cystadenomas and 4 cystadenocarcinomas at a large institution in Cleveland over the course of 17 years. Roughly 85% are reported in women, aged 41 to 53 years old.[4][5] The key clinical difference with cystadenomas compared to BDH is the chance that they will become malignant.
Polycystic Liver Disease
Polycystic liver diseases are genetic disorders that can be either liver-specific or part of a broader disease complex known as autosomal dominant polycystic kidney disease (ADPKD). The defects in pure polycystic liver disease are those of glycoprotein metabolism and thus the lesions are fluid-filled biliary epithelial cysts full of the byproducts of errant oligosaccharide metabolism.[6][7][8] There is recent evidence to suggest that the development of cysts is further exacerbated by a secretin mediated increase in cyclic AMP that generates increased biliary fluid prodcution[9]
The Autosomal Dominant Polycystic Kidney Disease is caused by two mutations in the ADPKD-1 gene on chromosome 16 and ADPKD-2 on chromosome 4. Polycystins, the product of these genes, are proteins expressed on the epithelium of renal tubules, hepatic bile ductules and pancreatic ducts that appear to play crucial roles in the mechanosensation of cilia. [10][11] In other words, these mutations limit the ability of the cells in these tissues to detect and react to structural distortions, leading to cystic degeneration.
Hydatid Cysts
Hydatid disease – Echinococcosis – is caused by the larval form of Echinococcus granulosus, has a worldwide distribution and is endemic in many developing countries. It is isolated to the liver in 50-70% of patients and the lungs in 20-30%.[12] If symptomatic, patients typically present with pain/discomfort, an abdominal mass, fever or, very rarely, cyst rupture.
References
- ↑ Tapper EB, Adsay NV, Kalb B, Martin D, Kooby D, Sarmiento JM. Symptomatic Bile Duct Hamartomas: Surgical Management in an MRI Driven Practice. J Gastrointest Surg. 2010 May 18. [Epub ahead of print]
- ↑ Regev A et al. Large Cystic Lesions of the Liver in Adults:A 15-Year Experience in a Tertiary Center. J Am Coll Surg 2001;193:36–45
- ↑ Gamblin TC, Holloway SE, Heckman JT, Geller DA. Laparoscopic resection of benign hepatic cysts: a new standard. J Am Coll Surg. Nov 2008;207(5):731-736.
- ↑ Devaney K, Goodman ZD, Ishak KG. Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients. Am J Pathol 1994;18:1078–1091.
- ↑ Vogt et al. Cystadenoma and Cystadenocarcinoma of the Liver:A Single Center Experience. J AmColl Surg 2005;200:727–733
- ↑ Torres VE. Treatment of polycystic liver disease: one size does not fit all. Am J Kidney Dis. 2007;49:725–728
- ↑ Davila S, Furu L, Gharavi AG, et al: Mutations in SEC63 cause autosomal dominant polycystic liver disease. Nat Genet 36:575-577, 2004
- ↑ Li A, Davila S, Furu L, et al: Mutations in PRKCSH cause isolated autosomal dominant polycystic liver disease. Am J Hum Genet 72:691-703, 2003
- ↑ Gong AY, Tietz PS, Muff MA, et al. Somatostatin stimulates ductal bile absorption and inhibits ductal bile secretion in mice via SSTR2 on cholangiocytes. Am J Physiol Cell Physiol 2003;284:C1205–C1214
- ↑ Geng L et al. Identification and Localization of Polycystin, the PKD1 Gene Product.J. Clin. Invest.1996. 98:2674–2682
- ↑ Nauli SM et al. Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells. Nature Genetics 2003;33: 129 – 137
- ↑ Ammann RW, Eckert J. Cestodes. Echinococcus. Gastroenterol Clin North Am 1996;25:655–689.
Pathophysiology
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References
Causes
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Causes
Most commonly, liver cysts are the result of malformed and dilated bile ducts, set in the background of fibrous stroma, thought to be constituents of the spectrum of fibropolycystic disease including congenital hepatic fibrosis, autosomal recessive polycystic kidney disease, bile duct atresia, Caroli’s disease, and mesenchymal hamartomas.[1] While the understanding of these lesions is evolving, there are three major categories: bile duct hamartomas, cystadenomas and cystedocarcinomas.
In addition there are hydatid cysts – Echinococcosis – which are caused by the larval form of Echinococcus granulosus.
References
- ↑ Brancatelli G, Federle MP, Vilgrain V, Vullierme MP, Marin D, Lagalla R. Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 2005;25(3):659-670
Differentiating Hepatic Cysts from other Diseases
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Differentiating Hepatic Cysts from other Diseases
- Aerobic gram negative bacteria
- Amebiasis
- Amebic cyst
- Bile duct hamartoma
- Clostridium Difficile
- Cystadenoma and Cystadenocarcinomas
- Echinococcal cyst
- Hydatid Cyst
- Inflammatory cyst
- Neisseria gonorrhea
- Neoplastic cyst
- Peliosis hepatitis
- Polycystic liver disease
- Retention cyst
- Solitary cyst
- Staphylococcal Infections
- Streptococcal Infections
- Syphilis
References
Epidemiology and Demographics
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References
Risk Factors
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References
Natural History, Complications and Prognosis
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References
Diagnosis
Diagnosis
Diagnostic Criteria | History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies
Treatment
Treatment
Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
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