eMedicine Specialties > Gastroenterology > Liver

Hepatic Cystadenomas: Differential Diagnoses & Workup

Author: Krishan Ariyarathna, MD, Staff Physician, Department of Internal Medicine, Creighton University Medical Center
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
Contributor Information and Disclosures

Updated: Nov 11, 2009

Differential Diagnoses

Hepatic Cysts
Hepatocellular Adenoma

Other Problems to Be Considered

Hepatic cystadenomas are considered in the differential diagnosis of other hepatic cystic lesions, including simple cysts, echinococcal cysts, and cystadenocarcinomas.1,2 Intracystic hemorrhage, septations, or mural nodularity can be present both in cystadenomas and in other cystic lesions of the liver. Less commonly, cystadenomas may be confused with necrotic neoplasms, cystic metastases, abscesses, cystic hamartomas, embryonal sarcomas, hematomas, or other congenital cysts. Diagnostic questions also may arise in patients with Caroli disease or other forms of polycystic liver disease. Multiple bile duct hamartomas (von Meyenburg complex) can also mimic biliary cystadenoma, both for presentation and for imaging.3

Workup

Laboratory Studies

  • Although liver test results may be normal in patients with biliary cystadenomas, elevation of alkaline phosphatase, bilirubin, and, less commonly, aminotransferase levels can be present.
  • Superinfection of the tumor may cause leukocytosis with a left shift. Anemia is extremely rare but theoretically possible secondary to bleeding.
  • Carbohydrate antigen (CA) 19-9 levels may be elevated in some cases. Carcinoembryonic antigen (CEA) and alpha-fetoprotein levels are usually normal.
  • Cyst fluid analysis at laparoscopy has been proposed in the surgical management of hepatic cysts. Elevated intracystic CA19-9 values were found in biliary cystadenomas compared to those of simple cysts.

Imaging Studies

  • Imaging studies are the key element of the workup.
  • On US, hepatic cystadenomas appear as anechoic lesions with internal septations. Focal hyperechoic areas within the lesion are common and can represent focal wall fibrosis, intracystic hemorrhage, or papillary projections.
  • On CT scan, the tumor appears as low-attenuation water density areas with focal enhancement after contrast administration. The septa and the mural nodules often are visualized. Involvement or compression of the portal vein and biliary tree can be appreciated best by CT scan.
  • MRI can help provide additional information about the nature of the cystic fluid (ie, hemorrhagic vs serous or mucinous). Lesions appear hyperintense on T2-weighted images and hypointense on T1-weighted images, sometimes with reduced perilesional rim signal intensity on T2-weighted images. Intracystic hemorrhage produces higher signal intensity on T1-weighted images than mucinous or bilious fluid content.
  • US is more sensitive in identifying internal septations, whereas CT scan provides anatomical relation to the liver.

Other Tests

  • Endoscopic retrograde cholangiopancreatography (ERCP) may demonstrate intraluminal filling defects or a cystic cavity communicating with the biliary tree. Apart from helping in the diagnosis of a cystadenocarcinoma, ERCP is also helpful in decompressing the biliary system in patients with biliary obstruction. 
  • Magnetic resonance cholangiopancreatography (MRCP) is an alternative to ERCP in the evaluation of pancreatic and biliary duct systems. Even though the resolution of MRCP is somewhat inferior to ERCP, the procedure is noninvasive and less expensive.

Procedures

  • Fine-needle aspiration biopsy
    • Initial imaging studies should be followed by fine-needle aspiration (FNA) biopsy of the liver, which may help provide important information about the nature of the lesion.
    • Cytology of the fluid or FNA of prominent papillary projections or wall nodules is useful in helping clarify the diagnosis, even though dissemination of malignant cells through the needle track is a theoretical concern.
    • The authors usually choose to proceed with FNA biopsy whenever a question exists regarding the diagnosis of a hepatic tumor. However, remember that needle biopsy findings from hepatic cystadenomas may be misleading because foci of adenocarcinomas can be easily missed.
    • If the diagnostic suspicion of a hepatic cystadenoma is high, a direct referral for surgical resection is indicated.
  • A laparoscopic approach with cyst fluid analysis for CA19-9 and CEA followed by cyst wall tissue sampling has been proposed. Elevated CA19-9 levels and premalignant or malignant histology should be followed by radical resection.

Histologic Findings

Solid hepatic lesions may be considered in the differential diagnosis, especially when the lesions appear irregular on imaging studies. Such lesions include FNH, adenomas, angiomyolipomas, and primary hepatic malignancies, such as hepatocellular carcinoma and cholangiocarcinoma.

A histological variant of biliary cystadenoma occurring primarily in women has been described as cystadenoma with mesenchymal stroma. This variant is characterized by the presence of spindle cells in the mesenchymal stroma that are capable of differentiating into different cell types, with a high premalignant potential.

Differentiation of cystadenomas from cystadenocarcinomas is particularly difficult. Imaging studies are not sensitive enough to completely exclude the presence of malignant degeneration in a cystadenoma. Determination of the tumor marker CA19-9 in the serum and in the cyst fluid has been suggested, but CA19-9 also can be expressed in the biliary epithelium lining of benign cystadenomas. For this reason, the presence of CA19-9 is not 100% reliable in the diagnosis of cystadenocarcinoma.

Embryonal sarcoma also is in the differential.

Cystadenomas may express a progesterone receptor in the mesenchymal cell component. Other markers demonstrated on immunohistochemistry are CA19-9, CEA, vimentin, and cytokeratin. In situ hybridization has demonstrated selective positivity for albumin messenger RNA in cystadenocarcinomas.

Available evidence shows that biliary cystadenomas tend to occur predominantly in women because these tumors are hormonally responsive.  This theory is further supported by immunohistochemical studies demonstrating positive estrogen/progesterone receptors associated with biliary cystadenomas.

More on Hepatic Cystadenomas

Overview: Hepatic Cystadenomas
Differential Diagnoses & Workup: Hepatic Cystadenomas
Treatment & Medication: Hepatic Cystadenomas
Follow-up: Hepatic Cystadenomas
References
Further Reading
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References

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  2. Choi HK, Lee JK, Lee KH, Lee KT, Rhee JC, Kim KH, et al. Differential Diagnosis for Intrahepatic Biliary Cystadenoma and Hepatic Simple Cyst: Significance of Cystic Fluid Analysis and Radiologic Findings. J Clin Gastroenterol. Sep 18 2009;[Medline].

  3. Zhang YL, Yuan L, Shen F, Wang Y. Hemorrhagic hepatic cysts mimicking biliary cystadenoma. World J Gastroenterol. Sep 28 2009;15(36):4601-3. [Medline].

  4. 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):731-6. [Medline].

  5. 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].

  6. 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].

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  8. 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].

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Further Reading

Clinical guideline

ACR Appropriateness Criteria® liver lesion characterization.
American College of Radiology - Medical Specialty Society. 1998 (revised 2006). 7 pages. NGC:005115


Clinical trial

Evaluation of Intra-operative Ultrasound Contrast Enhancement in the Evaluation of Liver Tumors


Related eMedicine topics
Biliary Cystadenoma/Cystadenocarcinoma

Cholangitis

Hepatic Cysts

Liver, Metastases

Bile Duct Tumors

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Keywords

hepatic cystadenoma, liver cyst, liver cysts, hepatic cyst, hepatic cysts, cyst on liver, cysts on liver, hepatic lesion, hepatic lesions, liver cyst symptoms, biliary cystadenoma, multilocular cystic tumor, cystic tumor, liver tumor, liver adenoma, liver cystadenoma, liver lesion, benign tumor, hepatic tumor

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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: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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.

CME Editor

Alex J Mechaber, MD, FACP, 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; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
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 and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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