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Mucinous Cystic Neoplasm of Liver
Cody Keller, MD; Aya Kamaya, MD, FSRU, FSAR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Mucinous cystic neoplasm (MCN)
          • Synonyms

            • Biliary mucinous cystic neoplasm
            • Hepatobiliary cystadenoma/cystadenocarcinoma, biliary cystic tumor, biliary cystic neoplasm
          • Definitions

            • Rare, unilocular or multilocular, cystic epithelial tumor with goblet cells and subepithelial, ovarian-like stroma

          IMAGING

          • General Features

            • Ultrasonographic Findings

              • Radiographic Findings

                • CT Findings

                  • MR 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. Boyum JH et al: Hepatic mucinous cystic neoplasm versus simple biliary cyst: assessment of distinguishing imaging features using CT and MRI. AJR Am J Roentgenol. 216(2):403-11, 2021
                                          2. Budkule DP et al: Infrequent intrahepatic cystic neoplasm: dilemmas in diagnosis and management. BMJ Case Rep. 12(5), 2019
                                          3. Kovacs MD et al: Differentiating biliary cystadenomas from benign hepatic cysts: preliminary analysis of new predictive imaging features. Clin Imaging. 49:44-7, 2018
                                          4. Kunovsky L et al: Mucinous cystic neoplasm of the liver or intraductal papillary mucinous neoplasm of the bile duct? A case report and a review of literature. Ann Hepatol. 17(3):519-24, 2018
                                          5. Lee DH et al: Primary malignant tumours in the non-cirrhotic liver. Eur J Radiol. 95:349-61, 2017
                                          6. Arnaoutakis DJ et al: Management of biliary cystic tumors: a multi-institutional analysis of a rare liver tumor. Ann Surg. 261(2):361-7, 2015
                                          7. Chen YW et al: Surgical management of biliary cystadenoma and cystadenocarcinoma of the liver. Genet Mol Res. 13(3):6383-90, 2014
                                          8. Cogley JR et al: MR imaging of benign focal liver lesions. Radiol Clin North Am. 52(4):657-82, 2014
                                          9. Kubota K et al: Clinicopathological features and prognosis of mucin-producing bile duct tumor and mucinous cystic tumor of the liver: a multi-institutional study by the Japan Biliary Association. J Hepatobiliary Pancreat Sci. 21(3):176-85, 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. Zen Y et al: Intraductal papillary neoplasms and mucinous cystic neoplasms of the hepatobiliary system: demographic differences between Asian and Western populations, and comparison with pancreatic counterparts. Histopathology. 65(2):164-73, 2014
                                          12. Bakoyiannis A et al: Rare cystic liver lesions: a diagnostic and managing challenge. World J Gastroenterol. 19(43):7603-19, 2013
                                          13. Lantinga MA et al: Evaluation of hepatic cystic lesions. World J Gastroenterol. 19(23):3543-54, 2013
                                          14. Macedo FI: Current management of noninfectious hepatic cystic lesions: a review of the literature. World J Hepatol. 5(9):462-9, 2013
                                          15. Qian LJ et al: Spectrum of multilocular cystic hepatic lesions: CT and MR imaging findings with pathologic correlation. Radiographics. 33(5):1419-33, 2013
                                          16. Xu HX et al: Imaging features of intrahepatic biliary cystadenoma and cystadenocarcinoma on B-mode and contrast-enhanced ultrasound. Ultraschall Med. 33(7):E241-9, 2012
                                          17. Emre A et al: Intrahepatic biliary cystic neoplasms: surgical results of 9 patients and literature review. World J Gastroenterol. 17(3):361-5, 2011
                                          18. 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
                                          19. Bosman FT et al: World Health Organization classification of tumours. Pathology and genetics of tumours of the digestive system. IARC Press, 2010
                                          20. 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
                                          21. Lam MM et al: Ovarian-type stroma in hepatobiliary cystadenomas and pancreatic mucinous cystic neoplasms: an immunohistochemical study. Am J Clin Pathol. 129(2):211-8, 2008
                                          22. Pojchamarnwiputh S et al: Computed tomography of biliary cystadenoma and biliary cystadenocarcinoma. Singapore Med J. 49(5):392-6, 2008
                                          23. Abdul-Al HM et al: Expression of estrogen and progesterone receptors and inhibin-alpha in hepatobiliary cystadenoma: an immunohistochemical study. Virchows Arch. 450(6):691-7, 2007
                                          24. Lewin M et al: Assessment of MRI and MRCP in diagnosis of biliary cystadenoma and cystadenocarcinoma. Eur Radiol. 16(2):407-13, 2006
                                          25. Teoh AY et al: Biliary cystadenoma and other complicated cystic lesions of the liver: diagnostic and therapeutic challenges. World J Surg. 30(8):1560-6, 2006
                                          26. Mortelé KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001
                                          27. 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
                                          28. Devaney K et al: Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients. Am J Surg Pathol 18:1078, 1994
                                          29. Korobkin M et al: Biliary cystadenoma and cystadenocarcinoma: CT and sonographic findings. AJR Am J Roentgenol. 153(3):507-11, 1989
                                          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

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Mucinous cystic neoplasm (MCN)
                                                  • Synonyms

                                                    • Biliary mucinous cystic neoplasm
                                                    • Hepatobiliary cystadenoma/cystadenocarcinoma, biliary cystic tumor, biliary cystic neoplasm
                                                  • Definitions

                                                    • Rare, unilocular or multilocular, cystic epithelial tumor with goblet cells and subepithelial, ovarian-like stroma

                                                  IMAGING

                                                  • General Features

                                                    • Ultrasonographic Findings

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • MR 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. Boyum JH et al: Hepatic mucinous cystic neoplasm versus simple biliary cyst: assessment of distinguishing imaging features using CT and MRI. AJR Am J Roentgenol. 216(2):403-11, 2021
                                                                                  2. Budkule DP et al: Infrequent intrahepatic cystic neoplasm: dilemmas in diagnosis and management. BMJ Case Rep. 12(5), 2019
                                                                                  3. Kovacs MD et al: Differentiating biliary cystadenomas from benign hepatic cysts: preliminary analysis of new predictive imaging features. Clin Imaging. 49:44-7, 2018
                                                                                  4. Kunovsky L et al: Mucinous cystic neoplasm of the liver or intraductal papillary mucinous neoplasm of the bile duct? A case report and a review of literature. Ann Hepatol. 17(3):519-24, 2018
                                                                                  5. Lee DH et al: Primary malignant tumours in the non-cirrhotic liver. Eur J Radiol. 95:349-61, 2017
                                                                                  6. Arnaoutakis DJ et al: Management of biliary cystic tumors: a multi-institutional analysis of a rare liver tumor. Ann Surg. 261(2):361-7, 2015
                                                                                  7. Chen YW et al: Surgical management of biliary cystadenoma and cystadenocarcinoma of the liver. Genet Mol Res. 13(3):6383-90, 2014
                                                                                  8. Cogley JR et al: MR imaging of benign focal liver lesions. Radiol Clin North Am. 52(4):657-82, 2014
                                                                                  9. Kubota K et al: Clinicopathological features and prognosis of mucin-producing bile duct tumor and mucinous cystic tumor of the liver: a multi-institutional study by the Japan Biliary Association. J Hepatobiliary Pancreat Sci. 21(3):176-85, 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. Zen Y et al: Intraductal papillary neoplasms and mucinous cystic neoplasms of the hepatobiliary system: demographic differences between Asian and Western populations, and comparison with pancreatic counterparts. Histopathology. 65(2):164-73, 2014
                                                                                  12. Bakoyiannis A et al: Rare cystic liver lesions: a diagnostic and managing challenge. World J Gastroenterol. 19(43):7603-19, 2013
                                                                                  13. Lantinga MA et al: Evaluation of hepatic cystic lesions. World J Gastroenterol. 19(23):3543-54, 2013
                                                                                  14. Macedo FI: Current management of noninfectious hepatic cystic lesions: a review of the literature. World J Hepatol. 5(9):462-9, 2013
                                                                                  15. Qian LJ et al: Spectrum of multilocular cystic hepatic lesions: CT and MR imaging findings with pathologic correlation. Radiographics. 33(5):1419-33, 2013
                                                                                  16. Xu HX et al: Imaging features of intrahepatic biliary cystadenoma and cystadenocarcinoma on B-mode and contrast-enhanced ultrasound. Ultraschall Med. 33(7):E241-9, 2012
                                                                                  17. Emre A et al: Intrahepatic biliary cystic neoplasms: surgical results of 9 patients and literature review. World J Gastroenterol. 17(3):361-5, 2011
                                                                                  18. 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
                                                                                  19. Bosman FT et al: World Health Organization classification of tumours. Pathology and genetics of tumours of the digestive system. IARC Press, 2010
                                                                                  20. 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
                                                                                  21. Lam MM et al: Ovarian-type stroma in hepatobiliary cystadenomas and pancreatic mucinous cystic neoplasms: an immunohistochemical study. Am J Clin Pathol. 129(2):211-8, 2008
                                                                                  22. Pojchamarnwiputh S et al: Computed tomography of biliary cystadenoma and biliary cystadenocarcinoma. Singapore Med J. 49(5):392-6, 2008
                                                                                  23. Abdul-Al HM et al: Expression of estrogen and progesterone receptors and inhibin-alpha in hepatobiliary cystadenoma: an immunohistochemical study. Virchows Arch. 450(6):691-7, 2007
                                                                                  24. Lewin M et al: Assessment of MRI and MRCP in diagnosis of biliary cystadenoma and cystadenocarcinoma. Eur Radiol. 16(2):407-13, 2006
                                                                                  25. Teoh AY et al: Biliary cystadenoma and other complicated cystic lesions of the liver: diagnostic and therapeutic challenges. World J Surg. 30(8):1560-6, 2006
                                                                                  26. Mortelé KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001
                                                                                  27. 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
                                                                                  28. Devaney K et al: Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients. Am J Surg Pathol 18:1078, 1994
                                                                                  29. Korobkin M et al: Biliary cystadenoma and cystadenocarcinoma: CT and sonographic findings. AJR Am J Roentgenol. 153(3):507-11, 1989