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Infantile Hemangioma, Musculoskeletal
A. Carlson Merrow, Jr., MD, FAAP
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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 higher grade vascular tumor)
              • Widespread misuse of "hemangioma" nomenclature in medical literature
            • Definitions

              • 2014 revised classification by the International Society for the Study of Vascular Anomalies (ISSVA) retains 2 main categories
                • Vascular tumors (true neoplasms with cellular proliferation; generally grow out of proportion to patient)
                  • Benign: Infantile hemangioma, 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; generally grow commensurate with patient)
                  • Simple: Venous, lymphatic, capillary, arteriovenous
                  • Combined: Capillary/venous/lymphatic, etc.
                  • Anomalies of major-named vessels (channel-type disorders)
                  • Syndromic [Klippel-Trenaunay, Parkes-Weber, Sturge-Weber, congenital lipomatous overgrowth with vascular malformations, epidermal nevi & spinal/skeletal anomalies (CLOVES), etc.]
              • 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 first 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)
                    • RICH mostly involuted by 14 months
              • Hemangiomatosis: Multifocal cutaneous IH + at least 1 extracutaneous site (usually liver)
            • Associated Syndromes

              • PHACE(S)
                • Posterior fossa malformations, segmental infantile Hemangioma, Arterial anomalies, Cardiac anomalies, Eye malformations, Sternal cleft/supraumbilical raphe
                  • Various cerebrovascular anomalies, aortic coarctation
                • Found in 1/3 of patients with segmental facial IH
              • 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

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic 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

                                          1. Hoornweg MJ et al: Malignant differential diagnosis in children referred for infantile hemangioma. Ann Plast Surg. 74(1): 43-6, 2015
                                          2. Wassef M et al: Vascular anomalies classification: recommendations from the international society for the study of vascular anomalies. Pediatrics. 136(1):e203-14, 2015
                                          3. Blei F et al: Current workup and therapy of infantile hemangiomas. Clin Dermatol. 32(4):459-70, 2014
                                          4. Püttgen KB: Diagnosis and management of infantile hemangiomas. Pediatr Clin North Am. 61(2):383-402, 2014
                                          5. Yilmaz S et al: Intramuscular capillary-type hemangioma: radiologic-pathologic correlation. Pediatr Radiol. 44(5):558-65, 2014
                                          6. Greenberger S et al: Pathogenesis of infantile haemangioma. Br J Dermatol. 169(1):12-9, 2013
                                          7. Bingham MM et al: Propranolol reduces infantile hemangioma volume and vessel density. Otolaryngol Head Neck Surg. 147(2):338-44, 2012
                                          8. Talaat AA et al: Propranolol treatment of infantile hemangioma: clinical and radiologic evaluations. J Pediatr Surg. 47(4):707-14, 2012
                                          9. 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
                                          10. 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
                                          11. Sidbury R: Update on vascular tumors of infancy. Curr Opin Pediatr. 22(4):432-7, 2010
                                          12. Moukaddam H et al: MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol. 38(6):535-47, 2009
                                          13. 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
                                          14. Konez O et al: Angiographic features of rapidly involuting congenital hemangioma (RICH). Pediatr Radiol. 33(1):15-9, 2003
                                          15. Dubois J et al: Vascular soft-tissue tumors in infancy: distinguishing features on Doppler sonography. AJR Am J Roentgenol. 178(6):1541-5, 2002
                                          16. Laor T et al: Congenital anomalies and vascular birthmarks of the lower extremities. Magn Reson Imaging Clin N Am. 6(3):497-519, 1998
                                          17. 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
                                          Related Anatomy
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                                          Related Differential Diagnoses
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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 higher grade vascular tumor)
                                                      • Widespread misuse of "hemangioma" nomenclature in medical literature
                                                    • Definitions

                                                      • 2014 revised classification by the International Society for the Study of Vascular Anomalies (ISSVA) retains 2 main categories
                                                        • Vascular tumors (true neoplasms with cellular proliferation; generally grow out of proportion to patient)
                                                          • Benign: Infantile hemangioma, 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; generally grow commensurate with patient)
                                                          • Simple: Venous, lymphatic, capillary, arteriovenous
                                                          • Combined: Capillary/venous/lymphatic, etc.
                                                          • Anomalies of major-named vessels (channel-type disorders)
                                                          • Syndromic [Klippel-Trenaunay, Parkes-Weber, Sturge-Weber, congenital lipomatous overgrowth with vascular malformations, epidermal nevi & spinal/skeletal anomalies (CLOVES), etc.]
                                                      • 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 first 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)
                                                            • RICH mostly involuted by 14 months
                                                      • Hemangiomatosis: Multifocal cutaneous IH + at least 1 extracutaneous site (usually liver)
                                                    • Associated Syndromes

                                                      • PHACE(S)
                                                        • Posterior fossa malformations, segmental infantile Hemangioma, Arterial anomalies, Cardiac anomalies, Eye malformations, Sternal cleft/supraumbilical raphe
                                                          • Various cerebrovascular anomalies, aortic coarctation
                                                        • Found in 1/3 of patients with segmental facial IH
                                                      • 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

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • MR Findings

                                                            • Ultrasonographic 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

                                                                                  1. Hoornweg MJ et al: Malignant differential diagnosis in children referred for infantile hemangioma. Ann Plast Surg. 74(1): 43-6, 2015
                                                                                  2. Wassef M et al: Vascular anomalies classification: recommendations from the international society for the study of vascular anomalies. Pediatrics. 136(1):e203-14, 2015
                                                                                  3. Blei F et al: Current workup and therapy of infantile hemangiomas. Clin Dermatol. 32(4):459-70, 2014
                                                                                  4. Püttgen KB: Diagnosis and management of infantile hemangiomas. Pediatr Clin North Am. 61(2):383-402, 2014
                                                                                  5. Yilmaz S et al: Intramuscular capillary-type hemangioma: radiologic-pathologic correlation. Pediatr Radiol. 44(5):558-65, 2014
                                                                                  6. Greenberger S et al: Pathogenesis of infantile haemangioma. Br J Dermatol. 169(1):12-9, 2013
                                                                                  7. Bingham MM et al: Propranolol reduces infantile hemangioma volume and vessel density. Otolaryngol Head Neck Surg. 147(2):338-44, 2012
                                                                                  8. Talaat AA et al: Propranolol treatment of infantile hemangioma: clinical and radiologic evaluations. J Pediatr Surg. 47(4):707-14, 2012
                                                                                  9. 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
                                                                                  10. 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
                                                                                  11. Sidbury R: Update on vascular tumors of infancy. Curr Opin Pediatr. 22(4):432-7, 2010
                                                                                  12. Moukaddam H et al: MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol. 38(6):535-47, 2009
                                                                                  13. 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
                                                                                  14. Konez O et al: Angiographic features of rapidly involuting congenital hemangioma (RICH). Pediatr Radiol. 33(1):15-9, 2003
                                                                                  15. Dubois J et al: Vascular soft-tissue tumors in infancy: distinguishing features on Doppler sonography. AJR Am J Roentgenol. 178(6):1541-5, 2002
                                                                                  16. Laor T et al: Congenital anomalies and vascular birthmarks of the lower extremities. Magn Reson Imaging Clin N Am. 6(3):497-519, 1998
                                                                                  17. 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