Hepatic Cystadenomas 

  • Author: Krishan Ariyarathna, MD; Chief Editor: Julian Katz, MD   more...
 
Updated: Jan 4, 2012
 

Background

Hepatic (biliary) cystadenomas are rare multilocular cystic tumors of the liver that are derived from the biliary epithelium and are predominantly located in the right hepatic lobe. These tumors usually involve the hepatic parenchyma (approximately 85% of cases) and occasionally the extrahepatic biliary tract. The size of the tumor is variable and ranges from 1.5-15 cm in diameter; it weighs as much as 6000 grams.

In 1892, Keen reported the first case of hepatic cystadenoma, which now accounts for 5% of all cystic lesions of the liver.

Hepatic cystadenomas are benign tumors, but they have a high rate of recurrence and a potential for neoplastic transformation in approximately 10% of cases. Removing these tumors and making a proper diagnosis is important, rather than monitoring them as is appropriate for other common benign hepatic tumors, such as focal nodular hyperplasia (FNH), adenoma, and hemangioma.

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Pathophysiology

Hepatic cystadenomas appear as multilocular cystic lesions (rarely unilocular) that are surrounded by a smooth and thick fibrous capsule. The tumors contain numerous internal septations and intraluminal papillary projections, which are lined by mucous-secreting cuboidal or columnar biliary epithelium. This epithelium is sometimes surrounded by a dense mesenchymal stroma containing smooth-muscle cells. A loose layer of collagen-containing blood vessels, nerves, and bile ducts further surrounds this area. True connections with the biliary tree are rare.

Traditionally, cystadenomas are thought to originate from the biliary epithelium, possibly from a congenitally aberrant bile duct. Other possibilities are that the tumors arise directly from embryonic foregut cells or peribiliary endocrine cells.

Two types of hepatic cystadenomas are described pathologically, as follows: mucinous and serous. Mucinous cystadenoma is the predominant type (95% of cases) that occurs in women. They are located in the intrahepatic region (84%), the common bile duct (6%), the hepatic ducts (4%), and the gallbladder (2%).

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Epidemiology

Frequency

United States

The prevalence of hepatic cystadenomas is low, with fewer than 200 cases reported in the literature. These tumors account for a very small number of all hepatic tumors.

International

The true prevalence of hepatic cystadenomas in different areas of the world is unknown.

Mortality/Morbidity

Because of their premalignant potential, untreated lesions carry significant mortality. Some authors report a malignant transformation rate of as high as 20-30%. However, in most series, hepatic cystadenomas carry an extremely low risk of mortality and morbidity after proper surgical resection.[1]

Race

The true prevalence of hepatic cystadenomas in different races is unknown.

Sex

Most tumors (80-85%) occur in women. The etiology of cystadenoma is unclear, but hormonal involvement is possible.

Age

The peak frequency of hepatic cystadenomas is in patients aged 30-50 years (mean age, 41.7-53.4 y), with two thirds of cases occurring in patients aged 40 years or older.

These tumors may arise as early as the first or second decade of life; however, they are extremely rare in children.

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Contributor Information and Disclosures
Author

Krishan Ariyarathna, MD  Staff Physician, Department of Internal Medicine, Creighton University Medical Center

Krishan Ariyarathna, MD is a member of the following medical societies: American College of Physicians and American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Sandeep Mukherjee, MB, BCh, MPH, FRCPC  Associate Professor, Department of Internal Medicine, Section of Gastroenterology and Hepatology, University of Nebraska Medical Center; Consulting Staff, Section of Gastroenterology and Hepatology, Veteran Affairs Medical Center

Sandeep Mukherjee, MB, BCh, MPH, FRCPC is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Merck Honoraria Speaking and teaching; Ikaria Pharmaceuticals Honoraria Board membership

Specialty Editor Board

John Gunn Lee, MD  Director of Pancreaticobiliary Service, Associate Professor, Department of Internal Medicine, Division of Gastroenterology, University of California at Irvine School of Medicine

John Gunn Lee, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

BS Anand, MD  Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine

BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of the previous author and coauthors, Andrea Duchini, MD, John Goss, MD, Murat Kilic, MD, Philip Seu, MD, and Paul J Pockros, MD, to the development and writing of this article.

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