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Gastrointestinal
Diagnosis
Liver
Malignant Neoplasms
Biliary Cystadenocarcinoma
Biliary Cystadenocarcinoma
Atif Zaheer, MD; Michael P. Federle, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Bile duct cystadenocarcinoma, hepatobiliary cystadenoma

            • Definitions

              • Rare, malignant or premalignant, unilocular or multilocular, cystic tumor
                • May arise from intrahepatic bile ducts (IHBDs) within liver (common site)
                • Uncommonly from extrahepatic biliary tree or gallbladder

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Angiographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Nagtegaal ID et al: The 2019 WHO classification of tumours of the digestive system. Histopathology. 76(2):182-8, 2020
                                            2. Averbukh LD et al: Biliary mucinous cystadenoma: a review of the literature. J Clin Transl Hepatol. 7(2):149-53, 2019
                                            3. Mavilia MG et al: Differentiating cystic liver lesions: a review of imaging modalities, diagnosis and management. J Clin Transl Hepatol. 6(2):208-16, 2018
                                            4. Park HJ et al: Intraductal papillary neoplasm of the bile duct: clinical, imaging, and pathologic features. AJR Am J Roentgenol. 211(1):67-75, 2018
                                            5. Dong Y et al: Contrast enhanced ultrasound features of hepatic cystadenoma and hepatic cystadenocarcinoma. Scand J Gastroenterol. 52(3):365-72, 2017
                                            6. Pitchaimuthu M et al: Cystic lesions of the liver-a review. Curr Probl Surg. 54(10):514-42, 2017
                                            7. Arnaoutakis DJ et al: Management of biliary cystic tumors: a multi-institutional analysis of a rare liver tumor. Ann Surg. 261(2):361-7, 2014
                                            8. Chen YW et al: Surgical management of biliary cystadenoma and cystadenocarcinoma of the liver. Genet Mol Res. 13(3):6383-90, 2014
                                            9. Cogley JR et al: MR imaging of benign focal liver lesions. Radiol Clin North Am. 52(4):657-82, 2014
                                            10. Soares KC et al: Cystic neoplasms of the liver: biliary cystadenoma and cystadenocarcinoma. J Am Coll Surg. 218(1):119-28, 2014
                                            11. Doepker M et al: Biliary cystadenoma: case series and review of the literature. Am Surg. 77(4):505-6, 2011
                                            12. Romagnoli R et al: Liver transplantation for symptomatic centrohepatic biliary cystadenoma. Clin Res Hepatol Gastroenterol. 35(5):408-13, 2011
                                            13. Sang X et al: Hepatobiliary cystadenomas and cystadenocarcinomas: a report of 33 cases. Liver Int. 31(9):1337-44, 2011
                                            14. Bogert JN et al: Education and imaging. Hepatobiliary and pancreatic: mucinous cystadenoma of the biliary tree. J Gastroenterol Hepatol. 25(7):1332, 2010
                                            15. Choi HK et al: Differential diagnosis for intrahepatic biliary cystadenoma and hepatic simple cyst: significance of cystic fluid analysis and radiologic findings. J Clin Gastroenterol. 44(4):289-93, 2010
                                            16. Seo JK et al: Appropriate diagnosis of biliary cystic tumors: comparison with atypical hepatic simple cysts. Eur J Gastroenterol Hepatol. 22(8):989-96, 2010
                                            17. Yu J et al: Hepatobiliary mucinous cystadenoma and cystadenocarcinoma: report of six cases and review of the literature. Hepatogastroenterology. 57(99-100):451-5, 2010
                                            18. Limongelli P et al: Cystic tumors of the biliary tract: a complete excision is crucial. Int Surg. 94(2):136-40, 2009
                                            19. Del Poggio P et al: Cystic tumors of the liver: a practical approach. World J Gastroenterol. 14(23):3616-20, 2008
                                            20. Delis SG et al: Intrahepatic biliary cystadenoma: a need for radical resection. Eur J Gastroenterol Hepatol. 20(1):10-4, 2008
                                            21. Mourra N et al: Clinical challenges and images in GI. Extrahepatic biliary cystadenoma with mesenchymal stroma. Gastroenterology. 134(5):1295, 1637, 2008
                                            22. Levy AD et al: From the archives of the AFIP. Benign tumors and tumorlike lesions of the gallbladder and extrahepatic bile ducts: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 22(2):387-413, 2002
                                            23. Mortelé KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001
                                            24. Gabata T et al: Biliary cystadenoma with mesenchymal stroma of the liver: correlation between unusual MR appearance and pathologic findings. J Magn Reson Imaging. 8(2):503-4, 1998
                                            25. Singh Y et al: Multiloculated cystic liver lesions: radiologic-pathologic differential diagnosis. Radiographics. 17(1):219-24, 1997
                                            26. Buetow PC et al: Biliary cystadenoma and cystadenocarcinoma: clinical-imaging-pathologic correlations with emphasis on the importance of ovarian stroma. Radiology. 196(3):805-10, 1995
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Clinical Issues

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Synonyms

                                                        • Bile duct cystadenocarcinoma, hepatobiliary cystadenoma

                                                      • Definitions

                                                        • Rare, malignant or premalignant, unilocular or multilocular, cystic tumor
                                                          • May arise from intrahepatic bile ducts (IHBDs) within liver (common site)
                                                          • Uncommonly from extrahepatic biliary tree or gallbladder

                                                      IMAGING

                                                      • General Features

                                                        • CT Findings

                                                          • MR Findings

                                                            • Ultrasonographic Findings

                                                              • Angiographic Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Gross Pathologic & Surgical Features

                                                                        • Microscopic Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Nagtegaal ID et al: The 2019 WHO classification of tumours of the digestive system. Histopathology. 76(2):182-8, 2020
                                                                                      2. Averbukh LD et al: Biliary mucinous cystadenoma: a review of the literature. J Clin Transl Hepatol. 7(2):149-53, 2019
                                                                                      3. Mavilia MG et al: Differentiating cystic liver lesions: a review of imaging modalities, diagnosis and management. J Clin Transl Hepatol. 6(2):208-16, 2018
                                                                                      4. Park HJ et al: Intraductal papillary neoplasm of the bile duct: clinical, imaging, and pathologic features. AJR Am J Roentgenol. 211(1):67-75, 2018
                                                                                      5. Dong Y et al: Contrast enhanced ultrasound features of hepatic cystadenoma and hepatic cystadenocarcinoma. Scand J Gastroenterol. 52(3):365-72, 2017
                                                                                      6. Pitchaimuthu M et al: Cystic lesions of the liver-a review. Curr Probl Surg. 54(10):514-42, 2017
                                                                                      7. Arnaoutakis DJ et al: Management of biliary cystic tumors: a multi-institutional analysis of a rare liver tumor. Ann Surg. 261(2):361-7, 2014
                                                                                      8. Chen YW et al: Surgical management of biliary cystadenoma and cystadenocarcinoma of the liver. Genet Mol Res. 13(3):6383-90, 2014
                                                                                      9. Cogley JR et al: MR imaging of benign focal liver lesions. Radiol Clin North Am. 52(4):657-82, 2014
                                                                                      10. Soares KC et al: Cystic neoplasms of the liver: biliary cystadenoma and cystadenocarcinoma. J Am Coll Surg. 218(1):119-28, 2014
                                                                                      11. Doepker M et al: Biliary cystadenoma: case series and review of the literature. Am Surg. 77(4):505-6, 2011
                                                                                      12. Romagnoli R et al: Liver transplantation for symptomatic centrohepatic biliary cystadenoma. Clin Res Hepatol Gastroenterol. 35(5):408-13, 2011
                                                                                      13. Sang X et al: Hepatobiliary cystadenomas and cystadenocarcinomas: a report of 33 cases. Liver Int. 31(9):1337-44, 2011
                                                                                      14. Bogert JN et al: Education and imaging. Hepatobiliary and pancreatic: mucinous cystadenoma of the biliary tree. J Gastroenterol Hepatol. 25(7):1332, 2010
                                                                                      15. Choi HK et al: Differential diagnosis for intrahepatic biliary cystadenoma and hepatic simple cyst: significance of cystic fluid analysis and radiologic findings. J Clin Gastroenterol. 44(4):289-93, 2010
                                                                                      16. Seo JK et al: Appropriate diagnosis of biliary cystic tumors: comparison with atypical hepatic simple cysts. Eur J Gastroenterol Hepatol. 22(8):989-96, 2010
                                                                                      17. Yu J et al: Hepatobiliary mucinous cystadenoma and cystadenocarcinoma: report of six cases and review of the literature. Hepatogastroenterology. 57(99-100):451-5, 2010
                                                                                      18. Limongelli P et al: Cystic tumors of the biliary tract: a complete excision is crucial. Int Surg. 94(2):136-40, 2009
                                                                                      19. Del Poggio P et al: Cystic tumors of the liver: a practical approach. World J Gastroenterol. 14(23):3616-20, 2008
                                                                                      20. Delis SG et al: Intrahepatic biliary cystadenoma: a need for radical resection. Eur J Gastroenterol Hepatol. 20(1):10-4, 2008
                                                                                      21. Mourra N et al: Clinical challenges and images in GI. Extrahepatic biliary cystadenoma with mesenchymal stroma. Gastroenterology. 134(5):1295, 1637, 2008
                                                                                      22. Levy AD et al: From the archives of the AFIP. Benign tumors and tumorlike lesions of the gallbladder and extrahepatic bile ducts: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 22(2):387-413, 2002
                                                                                      23. Mortelé KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001
                                                                                      24. Gabata T et al: Biliary cystadenoma with mesenchymal stroma of the liver: correlation between unusual MR appearance and pathologic findings. J Magn Reson Imaging. 8(2):503-4, 1998
                                                                                      25. Singh Y et al: Multiloculated cystic liver lesions: radiologic-pathologic differential diagnosis. Radiographics. 17(1):219-24, 1997
                                                                                      26. Buetow PC et al: Biliary cystadenoma and cystadenocarcinoma: clinical-imaging-pathologic correlations with emphasis on the importance of ovarian stroma. Radiology. 196(3):805-10, 1995