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Congenital Hepatic Hemangioma
Paula J. Woodward, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Pathogenesis of vascular liver lesion is not completely understood leading to confusing terminology
                • Multiple terms used for what is likely same lesion
                • Infantile hemangioendothelioma most common term seen in literature
            • Definitions

              • International Society for Study of Vascular Anomalies now classifies fetal lesion as congenital hemangioma
                • True benign vascular neoplasm occurring in soft tissues or viscera, typically liver
                  • Not to be confused with cavernous hemangiomas observed in adulthood (misnomer), which are actually venous malformations, not tumors
                • Further classified on biologic behavior
                  • Rapidly involuting congenital hemangioma (RICH)
                    • Most common type in fetus
                  • Noninvoluting congenital hemangioma (NICH)
                • Infantile hemangioma is distinct histologic type, which occurs in infancy and is not present at birth

            IMAGING

            • Ultrasonographic Findings

              • MR Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Microscopic Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                Selected References

                                1. Langham MR Jr et al: Current management of neonatal liver tumors. Curr Pediatr Rev. 11(3):195-204, 2015
                                2. Nagaraj UD et al: Diagnostic imaging of fetal and neonatal abdominal and soft tissue tumors. Curr Pediatr Rev. 11(3):143-50, 2015
                                3. Dasgupta R et al: ISSVA classification. Semin Pediatr Surg. 23(4):158-61, 2014
                                4. Hsi Dickie B et al: Hepatic vascular tumors. Semin Pediatr Surg. 23(4):168-72, 2014
                                5. Liang MG et al: Infantile and congenital hemangiomas. Semin Pediatr Surg. 23(4):162-7, 2014
                                6. Aslan H et al: Prenatal diagnosis of a liver cavernous hemangioma. Fetal Pediatr Pathol. 32(5):341-5, 2013
                                7. Kulungowski AM et al: Lessons from a liver hemangioma registry: subtype classification. J Pediatr Surg. 47(1):165-70, 2012
                                8. Roebuck D et al: Rapidly involuting congenital haemangioma (RICH) of the liver. Pediatr Radiol. 42(3):308-14, 2012
                                9. Makin E et al: Fetal and neonatal liver tumours. Early Hum Dev. 86(10):637-42, 2010
                                10. Schmitz R et al: Antenatal diagnosis of a giant fetal hepatic hemangioma and treatment with maternal corticosteroid. Ultraschall Med. 2009 Jun;30(3):223-6. Epub 2009 Jun 8. English, German. Erratum in: Ultraschall Med. 30(3):226, 2009
                                11. Walsh MA et al: Kaposiform hemangioendothelioma presenting antenatally with a pericardial effusion. J Pediatr Hematol Oncol. 30(10):761-3, 2008
                                12. Isaacs H Jr: Fetal and neonatal hepatic tumors. J Pediatr Surg. 42(11):1797-803, 2007
                                13. Woodward PJ et al: From the archives of the AFIP: a comprehensive review of fetal tumors with pathologic correlation. Radiographics. 25(1):215-42, 2005
                                14. Martinez AE et al: Kaposiform hemangioendothelioma associated with nonimmune fetal hydrops. Arch Pathol Lab Med. 128(6):678-81, 2004
                                15. Meirowitz NB et al: Hepatic hemangioendothelioma: prenatal sonographic findings and evolution of the lesion. J Clin Ultrasound. 28(5):258-63, 2000
                                16. Mhanni AA et al: Fetal hepatic haemangioendothelioma: a new association with elevated maternal serum alpha-fetoprotein. Prenat Diagn. 20(5):432-5, 2000
                                17. Marton T et al: Multifocal hemangioendothelioma of the fetus and placenta. Hum Pathol. 28(7):866-9, 1997
                                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

                                          • Synonyms

                                            • Pathogenesis of vascular liver lesion is not completely understood leading to confusing terminology
                                              • Multiple terms used for what is likely same lesion
                                              • Infantile hemangioendothelioma most common term seen in literature
                                          • Definitions

                                            • International Society for Study of Vascular Anomalies now classifies fetal lesion as congenital hemangioma
                                              • True benign vascular neoplasm occurring in soft tissues or viscera, typically liver
                                                • Not to be confused with cavernous hemangiomas observed in adulthood (misnomer), which are actually venous malformations, not tumors
                                              • Further classified on biologic behavior
                                                • Rapidly involuting congenital hemangioma (RICH)
                                                  • Most common type in fetus
                                                • Noninvoluting congenital hemangioma (NICH)
                                              • Infantile hemangioma is distinct histologic type, which occurs in infancy and is not present at birth

                                          IMAGING

                                          • Ultrasonographic Findings

                                            • MR Findings

                                              • Imaging Recommendations

                                                DIFFERENTIAL DIAGNOSIS

                                                  PATHOLOGY

                                                  • General Features

                                                    • Microscopic Features

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              Selected References

                                                              1. Langham MR Jr et al: Current management of neonatal liver tumors. Curr Pediatr Rev. 11(3):195-204, 2015
                                                              2. Nagaraj UD et al: Diagnostic imaging of fetal and neonatal abdominal and soft tissue tumors. Curr Pediatr Rev. 11(3):143-50, 2015
                                                              3. Dasgupta R et al: ISSVA classification. Semin Pediatr Surg. 23(4):158-61, 2014
                                                              4. Hsi Dickie B et al: Hepatic vascular tumors. Semin Pediatr Surg. 23(4):168-72, 2014
                                                              5. Liang MG et al: Infantile and congenital hemangiomas. Semin Pediatr Surg. 23(4):162-7, 2014
                                                              6. Aslan H et al: Prenatal diagnosis of a liver cavernous hemangioma. Fetal Pediatr Pathol. 32(5):341-5, 2013
                                                              7. Kulungowski AM et al: Lessons from a liver hemangioma registry: subtype classification. J Pediatr Surg. 47(1):165-70, 2012
                                                              8. Roebuck D et al: Rapidly involuting congenital haemangioma (RICH) of the liver. Pediatr Radiol. 42(3):308-14, 2012
                                                              9. Makin E et al: Fetal and neonatal liver tumours. Early Hum Dev. 86(10):637-42, 2010
                                                              10. Schmitz R et al: Antenatal diagnosis of a giant fetal hepatic hemangioma and treatment with maternal corticosteroid. Ultraschall Med. 2009 Jun;30(3):223-6. Epub 2009 Jun 8. English, German. Erratum in: Ultraschall Med. 30(3):226, 2009
                                                              11. Walsh MA et al: Kaposiform hemangioendothelioma presenting antenatally with a pericardial effusion. J Pediatr Hematol Oncol. 30(10):761-3, 2008
                                                              12. Isaacs H Jr: Fetal and neonatal hepatic tumors. J Pediatr Surg. 42(11):1797-803, 2007
                                                              13. Woodward PJ et al: From the archives of the AFIP: a comprehensive review of fetal tumors with pathologic correlation. Radiographics. 25(1):215-42, 2005
                                                              14. Martinez AE et al: Kaposiform hemangioendothelioma associated with nonimmune fetal hydrops. Arch Pathol Lab Med. 128(6):678-81, 2004
                                                              15. Meirowitz NB et al: Hepatic hemangioendothelioma: prenatal sonographic findings and evolution of the lesion. J Clin Ultrasound. 28(5):258-63, 2000
                                                              16. Mhanni AA et al: Fetal hepatic haemangioendothelioma: a new association with elevated maternal serum alpha-fetoprotein. Prenat Diagn. 20(5):432-5, 2000
                                                              17. Marton T et al: Multifocal hemangioendothelioma of the fetus and placenta. Hum Pathol. 28(7):866-9, 1997