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