Introduction
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.
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%).
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.
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.
Clinical
History
Hepatic cystadenomas are often discovered incidentally at a routine physical examination or on imaging studies, such as ultrasound (US) or CT scan. Less frequently, nonspecific symptoms related to compression of a neighboring organ may be noted. Presenting symptoms depend on the size and the location of the lesion. The final diagnosis is made after surgical resection.
- Roughly 15% of patients present with small intrahepatic lesions, which are usually asymptomatic and discovered incidentally after screening ultrasonography.
- For larger lesions, the typical presentation is that of an expanding mass in the right upper quadrant accompanied by pain, nausea, vomiting, and, in selected cases, cholangitis and sepsis.
- Unusual presentations include obstructive jaundice, ascites secondary to portal vein compression, and intracystic hemorrhage. Acute abdomen is a rare presentation and may be caused by the rupture of the cystic wall, intra-peritoneal hemorrhage, or cyst torsion.
Physical
Physical examination findings from patients with hepatic cystadenomas are usually unremarkable unless the tumor has reached a significant size and causes compressive symptoms. Physical examination may reveal a palpable and tender mass in the right upper quadrant or epigastrium and, less commonly, hepatomegaly. Lower-extremity edema and/or signs of portal hypertension may occur in patients with lesions large enough to compress the portal vein or vena cava.
- Splenomegaly may be an indication of a compressive effect on the portal circulation, with development of portal hypertension. These patients also may develop ascites.
- Jaundice suggests obstruction of the biliary system. Rare extension of the lesion in a pedunculated fashion inside the biliary system has been described.
- Obstruction of the inferior vena cava may cause marked lower-extremity edema.
- In the rare occurrence of intraperitoneal rupture of the lesion, the patient may have signs typical of acute peritonitis.
Causes
- The etiology of hepatic cystadenomas is unknown.
- The resemblance of embryonic structures, such as the gallbladder and the biliary tree, originating from the foregut suggests that these lesions arise from ectopic remnants. The fact that these tumors have been described as early as the first decade of life supports this theory.
- Immunohistochemistry and electron microscopy studies have contradicted the possible origin of these tumors from ectopic ovarian tissue.
- Environmental factors may play a significant role. Most tumors appear later in life.
- The marked female preponderance suggests a role for hormonal influence.
More on Hepatic Cystadenomas |
 Overview: Hepatic Cystadenomas |
| Differential Diagnoses & Workup: Hepatic Cystadenomas |
| Treatment & Medication: Hepatic Cystadenomas |
| Follow-up: Hepatic Cystadenomas |
| References |
| Next Page » |
References
Abdul-Al HM, Makhlouf HR, Goodman ZD. Expression of estrogen and progesterone receptors and inhibin-alpha in hepatobiliary cystadenoma: an immunohistochemical study. Virchows Arch. Jun 2007;450(6):691-7. [Medline].
Baudin G, Novellas S, Buratti MS, Saint-Paul MC, Chevallier P, Gugenheim J, et al. Atypical MRI features of a biliary cystadenoma revealed by jaundice. Clin Imaging. Nov-Dec 2006;30(6):413-5. [Medline].
Catinis GE, Frey DJ, Skinner JW, Balart LA. Hepatic cystadenoma: an unusual presentation. Am J Gastroenterol. May 1998;93(5):827-9. [Medline].
D'Errico A, Deleonardi G, Fiorentino M, Scoazec JY, Grigioni WF. Diagnostic implications of albumin messenger RNA detection and cytokeratin pattern in benign hepatic lesions and biliary cystadenocarcinoma. Diagn Mol Pathol. Dec 1998;7(6):289-94. [Medline].
Dardik H, Glotzer P, Silver C. Congenital hepatic cyst causing jaundice: report of a case and analogies with respiratory malformation. Ann Surg. Apr 1964;159:585-92. [Medline].
Franko J, Nussbaum ML, Morris JB. Choledochal cyst cholangiocarcinoma arising from adenoma: case report and a review of the literature. Curr Surg. Jul-Aug 2006;63(4):281-4. [Medline].
Grayson W, Teare J, Myburgh JA, Paterson AC. Immunohistochemical demonstration of progesterone receptor in hepatobiliary cystadenoma with mesenchymal stroma. Histopathology. Nov 1996;29(5):461-3. [Medline].
Horsmans Y, Laka A, Gigot JF, Geubel AP. Serum and cystic fluid CA 19-9 determinations as a diagnostic help in liver cysts of uncertain nature. Liver. Aug 1996;16(4):255-7. [Medline].
Ishak KG, Willis GW, Cummins SD, Bullock AA. Biliary cystadenoma and cystadenocarcinoma: report of 14 cases and review of the literature. Cancer. Jan 1977;39(1):322-38. [Medline].
Jenkins RL, Johnson LB, Lewis WD. Surgical approach to benign liver tumors. Semin Liver Dis. May 1994;14(2):178-89. [Medline].
Kanamori H, Kawahara H, Oh S, Mine T, Osawa H, Murakami T, et al. A case of biliary cystadenocarcinoma with recurrent jaundice. Diagnostic evaluation of computed tomography. Cancer. Jun 1 1985;55(11):2722-4. [Medline].
Karahan OI, Kahriman G, Soyuer I, Ok E. Hepatic von Meyenburg complex simulating biliary cystadenocarcinoma. Clin Imaging. Jan-Feb 2007;31(1):50-3. [Medline].
Koffron A, Rao S, Ferrario M, Abecassis M. Intrahepatic biliary cystadenoma: role of cyst fluid analysis and surgical management in the laparoscopic era. Surgery. Oct 2004;136(4):926-36. [Medline].
Mills GC, Alperin JB, Trimmer KB. Studies on variant glucose-6-phosphate dehydrogenases: G6PD Fort Worth. Biochem Med. Jul 1975;13(3):264-75. [Medline].
Ramacciato G, Nigri GR, D'Angelo F, Aurello P, Bellagamba R, Colarossi C, et al. Emergency laparotomy for misdiagnosed biliary cystadenoma originating from caudate lobe. World J Surg Oncol. 2006;4:76. [Medline].
Sanchez H, Gagner M, Rossi RL, Jenkins RL, Lewis WD, Munson JL, et al. Surgical management of nonparasitic cystic liver disease. Am J Surg. Jan 1991;161(1):113-8; discussion 118-9. [Medline].
Shimada M, Takenaka K, Gion T, Fujiwara Y, Taguchi K, Kajiyama K, et al. Treatment strategy for patients with cystic lesions mimicking a liver tumor: a recent 10-year surgical experience in Japan. Arch Surg. Jun 1998;133(6):643-6. [Medline].
Teoh AY, Ng SS, Lee KF, Lai PB. Biliary cystadenoma and other complicated cystic lesions of the liver: diagnostic and therapeutic challenges. World J Surg. Aug 2006;30(8):1560-6. [Medline].
Terada T, Kitamura Y, Ohta T. Endocrine cells in hepatobiliary cystadenomas and cystadenocarcinomas. Virchows Arch. Jan 1997;430(1):37-40. [Medline].
Wheeler DA, Edmondson HA. Cystadenoma with mesenchymal stroma (CMS) in the liver and bile ducts. A clinicopathologic study of 17 cases, 4 with malignant change. Cancer. Sep 15 1985;56(6):1434-45. [Medline].
Woods GL. Biliary cystadenocarcinoma: Case report of hepatic malignancy originating in benign cystadenoma. Cancer. Jun 15 1981;47(12):2936-40. [Medline].
Further Reading
Keywords
biliary cystadenoma, multilocular cystic tumor, cystic tumor, liver tumor, liver adenoma, liver cystadenoma, benign tumor, hepatic tumor
