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Peripheral (Intrahepatic) Cholangiocarcinoma
Michael P. Federle, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Cholangiocarcinoma (CCA)
            • Synonyms

              • Cholangiocellular carcinoma, intrahepatic CCA
            • Definitions

              • CCA: Adenocarcinoma arising from bile duct epithelium (cholangiocytes)
              • Types based on anatomic location
                • Peripheral (intrahepatic) CCA
                  • Arising from intrahepatic bile ducts distal to 2nd-order branches
                • Perihilar CCA (Klatskin tumor)
                  • Originates from 1st-order branches of right and left hepatic ducts and bifurcation of both hepatic ducts
                • Distal CCA

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Angiographic Findings

                      • Nuclear Medicine Findings

                        • Other Modality Findings

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                • Staging, Grading, & Classification

                                  • Gross Pathologic & Surgical Features

                                    • Microscopic Features

                                      CLINICAL ISSUES

                                      • Presentation

                                        • Demographics

                                          • Natural History & Prognosis

                                            • Treatment

                                              DIAGNOSTIC CHECKLIST

                                              • Consider

                                                • Image Interpretation Pearls

                                                  Selected References

                                                  1. Mantripragada S et al: Cholangiocarcinoma - part 2, tumoral and nontumoral mimics and imaging features helpful in differentiation. Curr Probl Diagn Radiol. ePub, 2021
                                                  2. Valle JW et al: Biliary tract cancer. Lancet. 397(10272):428-44, 2021
                                                  3. Mamone G et al: The "peripheral wash-out sign" in hepatic malignant lesions. Abdom Radiol (NY). 44(8):2937-8, 2019
                                                  4. Min JH et al: Intrahepatic mass-forming cholangiocarcinoma: arterial enhancement patterns at MRI and prognosis. Radiology. 290(3):691-9, 2019
                                                  5. Rhee H et al: A proposal of imaging classification of intrahepatic mass-forming cholangiocarcinoma into ductal and parenchymal types: clinicopathologic significance. Eur Radiol. 29(6):3111-21, 2019
                                                  6. Lo EC et al: Hepatocellular carcinoma and intrahepatic cholangiocarcinoma: imaging for diagnosis, tumor response to treatment and liver response to Radiation. Semin Radiat Oncol. 28(4):267-6, 2018
                                                  7. Sammon J et al: MRI features of combined hepatocellular- cholangiocarcinoma versus mass forming intrahepatic cholangiocarcinoma. Cancer Imaging. 18(1):8, 2018
                                                  8. Seo N et al: Cross-sectional imaging of intrahepatic cholangiocarcinoma: development, growth, spread, and prognosis. AJR Am J Roentgenol. 209(2):W64-75, 2017
                                                  9. Zhao YJ et al: Differentiation of mass-forming intrahepatic cholangiocarcinoma from poorly differentiated hepatocellular carcinoma: based on the multivariate analysis of contrast-enhanced computed tomography findings. Abdom Radiol (NY). 41(5):978-89, 2016
                                                  10. Tsunematsu S et al: Intratumoral artery on contrast-enhanced computed tomography imaging: differentiating intrahepatic cholangiocarcinoma from poorly differentiated hepatocellular carcinoma. Abdom Imaging. 40(6):1492-9, 2015
                                                  11. Apisarnthanarak P et al: The CT appearances for differentiating of peripheral, mass-forming cholangiocarcinoma and liver meatastases from colorectal adenocarcinoma. J Med Assoc Thai. 97(4):415-22, 2014
                                                  12. Roth CG et al: Hepatocellular carcinoma and other hepatic malignancies: MR imaging. Radiol Clin North Am. 52(4):683-707, 2014
                                                  13. Hu XX et al: Retrospective analysis of prognostic factors after liver transplantation for intrahepatic cholangiocarcinoma in China: a single-center experience. Hepatogastroenterology. 58(109):1255-9, 2011
                                                  14. Jiang BG et al: Clinical parameters predicting survival duration after hepatectomy for intrahepatic cholangiocarcinoma. Can J Gastroenterol. 25(11):603-8, 2011
                                                  15. Li YY et al: Clinicopathological characteristics of intrahepatic cholangiocarcinoma in patients with cirrhosis. Hepatogastroenterology. 58(110-111):1787-90, 2011
                                                  16. Yamamoto M et al: Surgical outcomes of intrahepatic cholangiocarcinoma. Surg Today. 41(7):896-902, 2011
                                                  17. Senda Y et al: Value of multidetector row CT in the assessment of longitudinal extension of cholangiocarcinoma: correlation between MDCT and microscopic findings. World J Surg. 33(7):1459-67, 2009
                                                  18. Singal A et al: Role of liver transplantation in the treatment of cholangiocarcinoma. Expert Rev Anticancer Ther. 9(4):491-502, 2009
                                                  19. Menias CO et al: Mimics of cholangiocarcinoma: spectrum of disease. Radiographics. 28(4):1115-29, 2008
                                                  20. Asayama Y et al: Delayed-phase dynamic CT enhancement as a prognostic factor for mass-forming intrahepatic cholangiocarcinoma. Radiology. 238(1):150-5, 2006
                                                  21. Ebied O et al: Hepatocellular-cholangiocarcinoma: helical computed tomography findings in 30 patients. J Comput Assist Tomogr. 27(2):117-24, 2003
                                                  22. Lim JH: Cholangiocarcinoma: morphologic classification according to growth pattern and imaging findings. AJR Am J Roentgenol. 181(3):819-27, 2003
                                                  23. Maetani Y et al: MR imaging of intrahepatic cholangiocarcinoma with pathologic correlation. AJR Am J Roentgenol. 176(6):1499-507, 2001
                                                  24. Kim TK et al: Peripheral cholangiocarcinoma of the liver: two-phase spiral CT findings. Radiology. 204(2):539-43, 1997
                                                  25. Lacomis JM et al: Cholangiocarcinoma: delayed CT contrast enhancement patterns. Radiology. 203(1):98-104, 1997
                                                  26. Soyer P et al: Imaging of intrahepatic cholangiocarcinoma: 1. Peripheral cholangiocarcinoma. AJR Am J Roentgenol. 165(6):1427-31, 1995
                                                  Related Anatomy
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                                                  Related Differential Diagnoses
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                                                  References
                                                  Tables

                                                  Tables

                                                  KEY FACTS

                                                  • Terminology

                                                    • Imaging

                                                      • Top Differential Diagnoses

                                                        • Pathology

                                                          • Clinical Issues

                                                            TERMINOLOGY

                                                            • Abbreviations

                                                              • Cholangiocarcinoma (CCA)
                                                            • Synonyms

                                                              • Cholangiocellular carcinoma, intrahepatic CCA
                                                            • Definitions

                                                              • CCA: Adenocarcinoma arising from bile duct epithelium (cholangiocytes)
                                                              • Types based on anatomic location
                                                                • Peripheral (intrahepatic) CCA
                                                                  • Arising from intrahepatic bile ducts distal to 2nd-order branches
                                                                • Perihilar CCA (Klatskin tumor)
                                                                  • Originates from 1st-order branches of right and left hepatic ducts and bifurcation of both hepatic ducts
                                                                • Distal CCA

                                                            IMAGING

                                                            • General Features

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Ultrasonographic Findings

                                                                    • Angiographic Findings

                                                                      • Nuclear Medicine Findings

                                                                        • Other Modality Findings

                                                                          • Imaging Recommendations

                                                                            DIFFERENTIAL DIAGNOSIS

                                                                              PATHOLOGY

                                                                              • General Features

                                                                                • Staging, Grading, & Classification

                                                                                  • Gross Pathologic & Surgical Features

                                                                                    • Microscopic Features

                                                                                      CLINICAL ISSUES

                                                                                      • Presentation

                                                                                        • Demographics

                                                                                          • Natural History & Prognosis

                                                                                            • Treatment

                                                                                              DIAGNOSTIC CHECKLIST

                                                                                              • Consider

                                                                                                • Image Interpretation Pearls

                                                                                                  Selected References

                                                                                                  1. Mantripragada S et al: Cholangiocarcinoma - part 2, tumoral and nontumoral mimics and imaging features helpful in differentiation. Curr Probl Diagn Radiol. ePub, 2021
                                                                                                  2. Valle JW et al: Biliary tract cancer. Lancet. 397(10272):428-44, 2021
                                                                                                  3. Mamone G et al: The "peripheral wash-out sign" in hepatic malignant lesions. Abdom Radiol (NY). 44(8):2937-8, 2019
                                                                                                  4. Min JH et al: Intrahepatic mass-forming cholangiocarcinoma: arterial enhancement patterns at MRI and prognosis. Radiology. 290(3):691-9, 2019
                                                                                                  5. Rhee H et al: A proposal of imaging classification of intrahepatic mass-forming cholangiocarcinoma into ductal and parenchymal types: clinicopathologic significance. Eur Radiol. 29(6):3111-21, 2019
                                                                                                  6. Lo EC et al: Hepatocellular carcinoma and intrahepatic cholangiocarcinoma: imaging for diagnosis, tumor response to treatment and liver response to Radiation. Semin Radiat Oncol. 28(4):267-6, 2018
                                                                                                  7. Sammon J et al: MRI features of combined hepatocellular- cholangiocarcinoma versus mass forming intrahepatic cholangiocarcinoma. Cancer Imaging. 18(1):8, 2018
                                                                                                  8. Seo N et al: Cross-sectional imaging of intrahepatic cholangiocarcinoma: development, growth, spread, and prognosis. AJR Am J Roentgenol. 209(2):W64-75, 2017
                                                                                                  9. Zhao YJ et al: Differentiation of mass-forming intrahepatic cholangiocarcinoma from poorly differentiated hepatocellular carcinoma: based on the multivariate analysis of contrast-enhanced computed tomography findings. Abdom Radiol (NY). 41(5):978-89, 2016
                                                                                                  10. Tsunematsu S et al: Intratumoral artery on contrast-enhanced computed tomography imaging: differentiating intrahepatic cholangiocarcinoma from poorly differentiated hepatocellular carcinoma. Abdom Imaging. 40(6):1492-9, 2015
                                                                                                  11. Apisarnthanarak P et al: The CT appearances for differentiating of peripheral, mass-forming cholangiocarcinoma and liver meatastases from colorectal adenocarcinoma. J Med Assoc Thai. 97(4):415-22, 2014
                                                                                                  12. Roth CG et al: Hepatocellular carcinoma and other hepatic malignancies: MR imaging. Radiol Clin North Am. 52(4):683-707, 2014
                                                                                                  13. Hu XX et al: Retrospective analysis of prognostic factors after liver transplantation for intrahepatic cholangiocarcinoma in China: a single-center experience. Hepatogastroenterology. 58(109):1255-9, 2011
                                                                                                  14. Jiang BG et al: Clinical parameters predicting survival duration after hepatectomy for intrahepatic cholangiocarcinoma. Can J Gastroenterol. 25(11):603-8, 2011
                                                                                                  15. Li YY et al: Clinicopathological characteristics of intrahepatic cholangiocarcinoma in patients with cirrhosis. Hepatogastroenterology. 58(110-111):1787-90, 2011
                                                                                                  16. Yamamoto M et al: Surgical outcomes of intrahepatic cholangiocarcinoma. Surg Today. 41(7):896-902, 2011
                                                                                                  17. Senda Y et al: Value of multidetector row CT in the assessment of longitudinal extension of cholangiocarcinoma: correlation between MDCT and microscopic findings. World J Surg. 33(7):1459-67, 2009
                                                                                                  18. Singal A et al: Role of liver transplantation in the treatment of cholangiocarcinoma. Expert Rev Anticancer Ther. 9(4):491-502, 2009
                                                                                                  19. Menias CO et al: Mimics of cholangiocarcinoma: spectrum of disease. Radiographics. 28(4):1115-29, 2008
                                                                                                  20. Asayama Y et al: Delayed-phase dynamic CT enhancement as a prognostic factor for mass-forming intrahepatic cholangiocarcinoma. Radiology. 238(1):150-5, 2006
                                                                                                  21. Ebied O et al: Hepatocellular-cholangiocarcinoma: helical computed tomography findings in 30 patients. J Comput Assist Tomogr. 27(2):117-24, 2003
                                                                                                  22. Lim JH: Cholangiocarcinoma: morphologic classification according to growth pattern and imaging findings. AJR Am J Roentgenol. 181(3):819-27, 2003
                                                                                                  23. Maetani Y et al: MR imaging of intrahepatic cholangiocarcinoma with pathologic correlation. AJR Am J Roentgenol. 176(6):1499-507, 2001
                                                                                                  24. Kim TK et al: Peripheral cholangiocarcinoma of the liver: two-phase spiral CT findings. Radiology. 204(2):539-43, 1997
                                                                                                  25. Lacomis JM et al: Cholangiocarcinoma: delayed CT contrast enhancement patterns. Radiology. 203(1):98-104, 1997
                                                                                                  26. Soyer P et al: Imaging of intrahepatic cholangiocarcinoma: 1. Peripheral cholangiocarcinoma. AJR Am J Roentgenol. 165(6):1427-31, 1995