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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Infantile hemangioma (IH)
Synonyms
- Capillary hemangioma, strawberry hemangioma
- Not cavernous hemangioma (actually venous malformation)
- Not synovial hemangioma (actually venous malformation)
- Not hemangioendothelioma (actually intermediate-grade vascular tumor)
- Widespread misuse of term "hemangioma" persists in medical literature
Definitions
- 2018 revised classification by International Society for Study of Vascular Anomalies (ISSVA) retains 2 main categories
- Vascular tumors (true neoplasms with cellular proliferation that generally grow out of proportion to patient)
- Benign: IH, congenital hemangioma, tufted angioma, pyogenic granuloma, etc.
- Locally aggressive or borderline: Kaposiform hemangioendothelioma, etc.
- Malignant: Angiosarcoma, epithelioid hemangioendothelioma
- Vascular malformations (congenital errors of vessel development that generally grow commensurate with patient)
- Simple: Venous, lymphatic, capillary, arteriovenous
- Combined: Capillary/venous/lymphatic, etc.
- Anomalies of major named vessels
- Syndromic: Klippel-Trenaunay, Parkes-Weber, Sturge-Weber, congenital lipomatous overgrowth with vascular malformations, epidermal nevi & spinal/skeletal anomalies (CLOVES), etc.
- Vascular tumors (true neoplasms with cellular proliferation that generally grow out of proportion to patient)
- Hemangioma: Benign vascular neoplasm with predictable life cycle
- Infantile hemangioma (IH)
- Most common soft tissue tumor of childhood
- Usually absent at birth with rapid growth over 1st few weeks/months of life (proliferating phase) followed by spontaneous regression over subsequent months to years (involuting phase)
- Congenital hemangioma (CH)
- Fully developed at birth
- No further cellular proliferation
- Rapidly involuting (RICH) vs. noninvoluting (NICH) vs. partially involuting (PICH)
- RICH is mostly involuted by 14 months
- Fully developed at birth
- Infantile hemangioma (IH)
- Hemangiomatosis: Multifocal cutaneous IH + at least 1 extracutaneous site (usually liver)
- 2018 revised classification by International Society for Study of Vascular Anomalies (ISSVA) retains 2 main categories
Associated Syndromes
- PHACE(S)
- Posterior fossa malformations, segmental infantile Hemangioma, Arterial anomalies, Cardiovascular anomalies, Eye malformations, Sternal cleft/supraumbilical raphe
- Various cerebrovascular anomalies, aortic coarctation
- Found in 1/3 of patients with segmental facial IH
- Posterior fossa malformations, segmental infantile Hemangioma, Arterial anomalies, Cardiovascular anomalies, Eye malformations, Sternal cleft/supraumbilical raphe
- PELVIS/LUMBAR
- Perineal hemangiomas, External genital malformations, Lipomyelomeningocele, Vesicorenal anomalies, Imperforate anus, Skin tag
- Lower body hemangiomas, Urogenital anomalies & ulceration, Myelopathy, Bony deformities, Arterial & anorectal anomalies, Renal anomalies
- PHACE(S)
IMAGING
General Features
Radiographic Findings
Ultrasonographic Findings
MR Findings
CT Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
Immunohistochemical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Abu Ata N et al: Neonatal vascular anomalies manifesting as soft-tissue masses. Pediatr Radiol. ePub, 2021
- Abu Ata N et al: Imaging of vascular anomalies in the pediatric musculoskeletal system. Semin Roentgenol. 56(3):288-306, 2021
- Gong X et al: Conventional ultrasonography and elastography for the diagnosis of congenital and infantile hemangiomas. J Dermatol. 47(5):527-33, 2020
- Peterman CM et al: Clinical and radiological characteristics of patients with retroperitoneal infantile hemangiomas. Pediatr Dermatol. 36(6):823-9, 2019
- Saito M et al: Usefulness of diffusion-weighted magnetic resonance imaging using apparent diffusion coefficient values for diagnosis of infantile hemangioma. J Comput Assist Tomogr. 43(4):563-7, 2019
- Yu Z et al: Clinical and radiological outcomes of infantile hemangioma treated with oral propranolol: a long-term follow-up study. J Dermatol. 46(5):376-82, 2019
- He L et al: Spectral Doppler ultrasound for predicting long-term response to topical timolol in children with infantile hemangioma. J Clin Ultrasound. 45(8):480-7, 2017
- Johnson CM et al: Clinical and sonographic features of pediatric soft-tissue vascular anomalies part 1: classification, sonographic approach and vascular tumors. Pediatr Radiol. 47(9):1184-95, 2017
- Merrow AC et al: 2014 Revised classification of vascular lesions from the International Society for the Study of Vascular Anomalies: radiologic-pathologic update. Radiographics. 36(5):1494-516, 2016
- Hoornweg MJ et al: Malignant differential diagnosis in children referred for infantile hemangioma. Ann Plast Surg. 74(1): 43-6, 2015
- Wassef M et al: Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 136(1):e203-14, 2015
- Blei F et al: Current workup and therapy of infantile hemangiomas. Clin Dermatol. 32(4):459-70, 2014
- Püttgen KB: Diagnosis and management of infantile hemangiomas. Pediatr Clin North Am. 61(2):383-402, 2014
- Yilmaz S et al: Intramuscular capillary-type hemangioma: radiologic-pathologic correlation. Pediatr Radiol. 44(5):558-65, 2014
- Greenberger S et al: Pathogenesis of infantile haemangioma. Br J Dermatol. 169(1):12-9, 2013
- Bingham MM et al: Propranolol reduces infantile hemangioma volume and vessel density. Otolaryngol Head Neck Surg. 147(2):338-44, 2012
- Talaat AA et al: Propranolol treatment of infantile hemangioma: clinical and radiologic evaluations. J Pediatr Surg. 47(4):707-14, 2012
- Restrepo R et al: Hemangiomas revisited: the useful, the unusual and the new. Part 1: overview and clinical and imaging characteristics. Pediatr Radiol. 41(7):895-904, 2011
- Restrepo R et al: Hemangiomas revisited: the useful, the unusual and the new. Part 2: endangering hemangiomas and treatment. Pediatr Radiol. 41(7):905-15, 2011
- Sidbury R: Update on vascular tumors of infancy. Curr Opin Pediatr. 22(4):432-7, 2010
- Gorincour G et al: Imaging characteristics of two subtypes of congenital hemangiomas: rapidly involuting congenital hemangiomas and non-involuting congenital hemangiomas. Pediatr Radiol. 35(12):1178-85, 2005
- Konez O et al: Angiographic features of rapidly involuting congenital hemangioma (RICH). Pediatr Radiol. 33(1):15-9, 2003
- Dubois J et al: Vascular soft-tissue tumors in infancy: distinguishing features on Doppler sonography. AJR Am J Roentgenol. 178(6):1541-5, 2002
- Laor T et al: Congenital anomalies and vascular birthmarks of the lower extremities. Magn Reson Imaging Clin N Am. 6(3):497-519, 1998
- Mulliken JB et al: Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 69(3):412-22, 1982
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Infantile hemangioma (IH)
Synonyms
- Capillary hemangioma, strawberry hemangioma
- Not cavernous hemangioma (actually venous malformation)
- Not synovial hemangioma (actually venous malformation)
- Not hemangioendothelioma (actually intermediate-grade vascular tumor)
- Widespread misuse of term "hemangioma" persists in medical literature
Definitions
- 2018 revised classification by International Society for Study of Vascular Anomalies (ISSVA) retains 2 main categories
- Vascular tumors (true neoplasms with cellular proliferation that generally grow out of proportion to patient)
- Benign: IH, congenital hemangioma, tufted angioma, pyogenic granuloma, etc.
- Locally aggressive or borderline: Kaposiform hemangioendothelioma, etc.
- Malignant: Angiosarcoma, epithelioid hemangioendothelioma
- Vascular malformations (congenital errors of vessel development that generally grow commensurate with patient)
- Simple: Venous, lymphatic, capillary, arteriovenous
- Combined: Capillary/venous/lymphatic, etc.
- Anomalies of major named vessels
- Syndromic: Klippel-Trenaunay, Parkes-Weber, Sturge-Weber, congenital lipomatous overgrowth with vascular malformations, epidermal nevi & spinal/skeletal anomalies (CLOVES), etc.
- Vascular tumors (true neoplasms with cellular proliferation that generally grow out of proportion to patient)
- Hemangioma: Benign vascular neoplasm with predictable life cycle
- Infantile hemangioma (IH)
- Most common soft tissue tumor of childhood
- Usually absent at birth with rapid growth over 1st few weeks/months of life (proliferating phase) followed by spontaneous regression over subsequent months to years (involuting phase)
- Congenital hemangioma (CH)
- Fully developed at birth
- No further cellular proliferation
- Rapidly involuting (RICH) vs. noninvoluting (NICH) vs. partially involuting (PICH)
- RICH is mostly involuted by 14 months
- Fully developed at birth
- Infantile hemangioma (IH)
- Hemangiomatosis: Multifocal cutaneous IH + at least 1 extracutaneous site (usually liver)
- 2018 revised classification by International Society for Study of Vascular Anomalies (ISSVA) retains 2 main categories
Associated Syndromes
- PHACE(S)
- Posterior fossa malformations, segmental infantile Hemangioma, Arterial anomalies, Cardiovascular anomalies, Eye malformations, Sternal cleft/supraumbilical raphe
- Various cerebrovascular anomalies, aortic coarctation
- Found in 1/3 of patients with segmental facial IH
- Posterior fossa malformations, segmental infantile Hemangioma, Arterial anomalies, Cardiovascular anomalies, Eye malformations, Sternal cleft/supraumbilical raphe
- PELVIS/LUMBAR
- Perineal hemangiomas, External genital malformations, Lipomyelomeningocele, Vesicorenal anomalies, Imperforate anus, Skin tag
- Lower body hemangiomas, Urogenital anomalies & ulceration, Myelopathy, Bony deformities, Arterial & anorectal anomalies, Renal anomalies
- PHACE(S)
IMAGING
General Features
Radiographic Findings
Ultrasonographic Findings
MR Findings
CT Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
Immunohistochemical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Abu Ata N et al: Neonatal vascular anomalies manifesting as soft-tissue masses. Pediatr Radiol. ePub, 2021
- Abu Ata N et al: Imaging of vascular anomalies in the pediatric musculoskeletal system. Semin Roentgenol. 56(3):288-306, 2021
- Gong X et al: Conventional ultrasonography and elastography for the diagnosis of congenital and infantile hemangiomas. J Dermatol. 47(5):527-33, 2020
- Peterman CM et al: Clinical and radiological characteristics of patients with retroperitoneal infantile hemangiomas. Pediatr Dermatol. 36(6):823-9, 2019
- Saito M et al: Usefulness of diffusion-weighted magnetic resonance imaging using apparent diffusion coefficient values for diagnosis of infantile hemangioma. J Comput Assist Tomogr. 43(4):563-7, 2019
- Yu Z et al: Clinical and radiological outcomes of infantile hemangioma treated with oral propranolol: a long-term follow-up study. J Dermatol. 46(5):376-82, 2019
- He L et al: Spectral Doppler ultrasound for predicting long-term response to topical timolol in children with infantile hemangioma. J Clin Ultrasound. 45(8):480-7, 2017
- Johnson CM et al: Clinical and sonographic features of pediatric soft-tissue vascular anomalies part 1: classification, sonographic approach and vascular tumors. Pediatr Radiol. 47(9):1184-95, 2017
- Merrow AC et al: 2014 Revised classification of vascular lesions from the International Society for the Study of Vascular Anomalies: radiologic-pathologic update. Radiographics. 36(5):1494-516, 2016
- Hoornweg MJ et al: Malignant differential diagnosis in children referred for infantile hemangioma. Ann Plast Surg. 74(1): 43-6, 2015
- Wassef M et al: Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 136(1):e203-14, 2015
- Blei F et al: Current workup and therapy of infantile hemangiomas. Clin Dermatol. 32(4):459-70, 2014
- Püttgen KB: Diagnosis and management of infantile hemangiomas. Pediatr Clin North Am. 61(2):383-402, 2014
- Yilmaz S et al: Intramuscular capillary-type hemangioma: radiologic-pathologic correlation. Pediatr Radiol. 44(5):558-65, 2014
- Greenberger S et al: Pathogenesis of infantile haemangioma. Br J Dermatol. 169(1):12-9, 2013
- Bingham MM et al: Propranolol reduces infantile hemangioma volume and vessel density. Otolaryngol Head Neck Surg. 147(2):338-44, 2012
- Talaat AA et al: Propranolol treatment of infantile hemangioma: clinical and radiologic evaluations. J Pediatr Surg. 47(4):707-14, 2012
- Restrepo R et al: Hemangiomas revisited: the useful, the unusual and the new. Part 1: overview and clinical and imaging characteristics. Pediatr Radiol. 41(7):895-904, 2011
- Restrepo R et al: Hemangiomas revisited: the useful, the unusual and the new. Part 2: endangering hemangiomas and treatment. Pediatr Radiol. 41(7):905-15, 2011
- Sidbury R: Update on vascular tumors of infancy. Curr Opin Pediatr. 22(4):432-7, 2010
- Gorincour G et al: Imaging characteristics of two subtypes of congenital hemangiomas: rapidly involuting congenital hemangiomas and non-involuting congenital hemangiomas. Pediatr Radiol. 35(12):1178-85, 2005
- Konez O et al: Angiographic features of rapidly involuting congenital hemangioma (RICH). Pediatr Radiol. 33(1):15-9, 2003
- Dubois J et al: Vascular soft-tissue tumors in infancy: distinguishing features on Doppler sonography. AJR Am J Roentgenol. 178(6):1541-5, 2002
- Laor T et al: Congenital anomalies and vascular birthmarks of the lower extremities. Magn Reson Imaging Clin N Am. 6(3):497-519, 1998
- Mulliken JB et al: Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 69(3):412-22, 1982
