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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
                • Hemangioendothelioma often seen in literature but is actually more aggressive vascular tumor typically presenting in adulthood
            • 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 subtype
                  • Partially involuting congenital hemangioma (PICH)
                  • Noninvoluting congenital hemangioma (NICH)
                • Infantile hemangioma is distinct entity, which develops in 1st few weeks of life but is rarely present at birth
                  • Multifocal with small to moderate-sized nodules

            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. Triana P et al: Congenital hepatic hemangiomas: clinical, histologic, and genetic correlation. J Pediatr Surg. 55(10):2170-6, 2020
                                2. Chaturvedi A et al: Ultrasound with Doppler evaluation of congenital hepatic vascular shunts. Pediatr Radiol. 48(11):1658-71, 2018
                                3. Iacobas I et al: Guidance document for hepatic hemangioma (infantile and congenital) evaluation and monitoring. J Pediatr. 203:294-300.e2, 2018
                                4. International Society for the Study of Vascular Anomalies: 2018 Classification. Accessed May 2, 2021. https://www.issva.org/classification
                                5. Langham MR Jr et al: Current management of neonatal liver tumors. Curr Pediatr Rev. 11(3):195-204, 2015
                                6. Nagaraj UD et al: Diagnostic imaging of fetal and neonatal abdominal and soft tissue tumors. Curr Pediatr Rev. 11(3):143-50, 2015
                                7. Liang MG et al: Infantile and congenital hemangiomas. Semin Pediatr Surg. 23(4):162-7, 2014
                                8. Aslan H et al: Prenatal diagnosis of a liver cavernous hemangioma. Fetal Pediatr Pathol. 32(5):341-5, 2013
                                9. Adeyiga AO et al: Focal hepatic masses in pediatric patients. AJR Am J Roentgenol. 199(4):W422-40, 2012
                                10. Kulungowski AM et al: Lessons from a liver hemangioma registry: subtype classification. J Pediatr Surg. 47(1):165-70, 2012
                                11. Roebuck D et al: Rapidly involuting congenital haemangioma (RICH) of the liver. Pediatr Radiol. 42(3):308-14, 2012
                                12. Makin E et al: Fetal and neonatal liver tumours. Early Hum Dev. 86(10):637-42, 2010
                                13. 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
                                14. Isaacs H Jr: Fetal and neonatal hepatic tumors. J Pediatr Surg. 42(11):1797-803, 2007
                                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
                                              • Hemangioendothelioma often seen in literature but is actually more aggressive vascular tumor typically presenting in adulthood
                                          • 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 subtype
                                                • Partially involuting congenital hemangioma (PICH)
                                                • Noninvoluting congenital hemangioma (NICH)
                                              • Infantile hemangioma is distinct entity, which develops in 1st few weeks of life but is rarely present at birth
                                                • Multifocal with small to moderate-sized nodules

                                          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. Triana P et al: Congenital hepatic hemangiomas: clinical, histologic, and genetic correlation. J Pediatr Surg. 55(10):2170-6, 2020
                                                              2. Chaturvedi A et al: Ultrasound with Doppler evaluation of congenital hepatic vascular shunts. Pediatr Radiol. 48(11):1658-71, 2018
                                                              3. Iacobas I et al: Guidance document for hepatic hemangioma (infantile and congenital) evaluation and monitoring. J Pediatr. 203:294-300.e2, 2018
                                                              4. International Society for the Study of Vascular Anomalies: 2018 Classification. Accessed May 2, 2021. https://www.issva.org/classification
                                                              5. Langham MR Jr et al: Current management of neonatal liver tumors. Curr Pediatr Rev. 11(3):195-204, 2015
                                                              6. Nagaraj UD et al: Diagnostic imaging of fetal and neonatal abdominal and soft tissue tumors. Curr Pediatr Rev. 11(3):143-50, 2015
                                                              7. Liang MG et al: Infantile and congenital hemangiomas. Semin Pediatr Surg. 23(4):162-7, 2014
                                                              8. Aslan H et al: Prenatal diagnosis of a liver cavernous hemangioma. Fetal Pediatr Pathol. 32(5):341-5, 2013
                                                              9. Adeyiga AO et al: Focal hepatic masses in pediatric patients. AJR Am J Roentgenol. 199(4):W422-40, 2012
                                                              10. Kulungowski AM et al: Lessons from a liver hemangioma registry: subtype classification. J Pediatr Surg. 47(1):165-70, 2012
                                                              11. Roebuck D et al: Rapidly involuting congenital haemangioma (RICH) of the liver. Pediatr Radiol. 42(3):308-14, 2012
                                                              12. Makin E et al: Fetal and neonatal liver tumours. Early Hum Dev. 86(10):637-42, 2010
                                                              13. 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
                                                              14. Isaacs H Jr: Fetal and neonatal hepatic tumors. J Pediatr Surg. 42(11):1797-803, 2007