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Musculoskeletal
Soft Tissue Masses
Kaposiform Hemangioendothelioma
Kaposiform Hemangioendothelioma
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        • Diagnostic Checklist

          TERMINOLOGY

          • Definitions

            • Kaposiform hemangioendothelioma (KHE): Aggressive, locally invasive vascular neoplasm of spindled endothelial cells & abnormal lymphatics
              • Not equivalent to hemangioma

          IMAGING

          • General Features

            • Imaging Recommendations

              DIFFERENTIAL DIAGNOSIS

                PATHOLOGY

                • General Features

                  • Microscopic Features

                    CLINICAL ISSUES

                    • Presentation

                      • Demographics

                        • Natural History & Prognosis

                          • Treatment

                            DIAGNOSTIC CHECKLIST

                            • Consider

                              Selected References

                              1. Abu Ata N et al: Neonatal vascular anomalies manifesting as soft-tissue masses. Pediatr Radiol. ePub, 2021
                              2. Abu Ata N et al: Imaging of vascular anomalies in the pediatric musculoskeletal system. Semin Roentgenol. 56(3):288-306, 2021
                              3. Peng S et al: Kaposiform haemangioendothelioma: magnetic resonance imaging features in 64 cases. BMC Pediatr. 21(1):107, 2021
                              4. Mansfield SA et al: Vascular tumors. Semin Pediatr Surg. 29(5):150975, 2020
                              5. Adams DM et al: Vascular anomalies: diagnosis of complicated anomalies and new medical treatment options. Hematol Oncol Clin North Am. 33(3):455-70, 2019
                              6. Gong X et al: Ultrasonography and magnetic resonance imaging features of kaposiform hemangioendothelioma and tufted angioma. J Dermatol. 46(10):835-42, 2019
                              7. Ji Y et al: Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences. Orphanet J Rare Dis. 14(1):165, 2019
                              8. Hu PA et al: Clinical and imaging features of kaposiform hemangioendothelioma. Br J Radiol. 91(1086):20170798, 2018
                              9. Ji Y et al: Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon: clinical characteristics and management. Cancer Manag Res. 10:3325-31, 2018
                              10. Schmid I et al: Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. World J Pediatr. 14(4):322-9, 2018
                              11. Putra J et al: Kaposiform haemangioendothelioma: a review with emphasis on histological differential diagnosis. Pathology. 49(4):356-62, 2017
                              12. Ryu YJ et al: Imaging findings of kaposiform hemangioendothelioma in children. Eur J Radiol. 86:198-205, 2017
                              13. Blei F: Kaposiform hemangioendothelioma: therapeutic efficacy for an enigmatic diagnosis. Pediatr Blood Cancer. 62(4):551-2, 2015
                              14. Iacobas I et al: Decreased vascularization of retroperitoneal kaposiform hemangioendothelioma induced by treatment with sirolimus explains relief of symptoms. Clin Imaging. 39(3):529-32, 2015
                              15. Wang Z et al: Successful treatment of Kasabach-Merritt phenomenon arising from kaposiform hemangioendothelioma by sirolimus. J Pediatr Hematol Oncol. 37(1):72-3, 2015
                              16. Adams DM et al: Other vascular tumors. Semin Pediatr Surg. 23(4):173-7, 2014
                              17. Croteau SE et al: Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr. 162(1):142-7, 2013
                              18. Drolet BA et al: Consensus-derived practice standards plan for complicated kaposiform hemangioendothelioma. J Pediatr. 163(1):285-91, 2013
                              19. Fernández Y et al: Kaposiform hemangioendothelioma. Eur J Intern Med. 20(2):106-13, 2009
                              20. Navarro OM et al: Pediatric soft-tissue tumors and pseudotumors: MR imaging features with pathologic correlation: part 1. Imaging approach, pseudotumors, vascular lesions, and adipocytic tumors. Radiographics. 29(3):887-906, 2009
                              21. Enjolras O et al: Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol. 42(2 Pt 1):225-35, 2000
                              22. Beaubien ER et al: Kaposiform hemangioendothelioma: a locally aggressive vascular tumor. J Am Acad Dermatol. 38(5 Pt 2):799-802, 1998
                              23. Enjolras O et al: Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas. J Pediatr. 130(4):631-40, 1997
                              24. Zukerberg LR et al: Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol. 17(4):321-8, 1993
                              Related Anatomy
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                              Related Differential Diagnoses
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                              References
                              Tables

                              Tables

                              KEY FACTS

                              • Terminology

                                • Imaging

                                  • Clinical Issues

                                    • Diagnostic Checklist

                                      TERMINOLOGY

                                      • Definitions

                                        • Kaposiform hemangioendothelioma (KHE): Aggressive, locally invasive vascular neoplasm of spindled endothelial cells & abnormal lymphatics
                                          • Not equivalent to hemangioma

                                      IMAGING

                                      • General Features

                                        • Imaging Recommendations

                                          DIFFERENTIAL DIAGNOSIS

                                            PATHOLOGY

                                            • General Features

                                              • Microscopic Features

                                                CLINICAL ISSUES

                                                • Presentation

                                                  • Demographics

                                                    • Natural History & Prognosis

                                                      • Treatment

                                                        DIAGNOSTIC CHECKLIST

                                                        • Consider

                                                          Selected References

                                                          1. Abu Ata N et al: Neonatal vascular anomalies manifesting as soft-tissue masses. Pediatr Radiol. ePub, 2021
                                                          2. Abu Ata N et al: Imaging of vascular anomalies in the pediatric musculoskeletal system. Semin Roentgenol. 56(3):288-306, 2021
                                                          3. Peng S et al: Kaposiform haemangioendothelioma: magnetic resonance imaging features in 64 cases. BMC Pediatr. 21(1):107, 2021
                                                          4. Mansfield SA et al: Vascular tumors. Semin Pediatr Surg. 29(5):150975, 2020
                                                          5. Adams DM et al: Vascular anomalies: diagnosis of complicated anomalies and new medical treatment options. Hematol Oncol Clin North Am. 33(3):455-70, 2019
                                                          6. Gong X et al: Ultrasonography and magnetic resonance imaging features of kaposiform hemangioendothelioma and tufted angioma. J Dermatol. 46(10):835-42, 2019
                                                          7. Ji Y et al: Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences. Orphanet J Rare Dis. 14(1):165, 2019
                                                          8. Hu PA et al: Clinical and imaging features of kaposiform hemangioendothelioma. Br J Radiol. 91(1086):20170798, 2018
                                                          9. Ji Y et al: Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon: clinical characteristics and management. Cancer Manag Res. 10:3325-31, 2018
                                                          10. Schmid I et al: Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. World J Pediatr. 14(4):322-9, 2018
                                                          11. Putra J et al: Kaposiform haemangioendothelioma: a review with emphasis on histological differential diagnosis. Pathology. 49(4):356-62, 2017
                                                          12. Ryu YJ et al: Imaging findings of kaposiform hemangioendothelioma in children. Eur J Radiol. 86:198-205, 2017
                                                          13. Blei F: Kaposiform hemangioendothelioma: therapeutic efficacy for an enigmatic diagnosis. Pediatr Blood Cancer. 62(4):551-2, 2015
                                                          14. Iacobas I et al: Decreased vascularization of retroperitoneal kaposiform hemangioendothelioma induced by treatment with sirolimus explains relief of symptoms. Clin Imaging. 39(3):529-32, 2015
                                                          15. Wang Z et al: Successful treatment of Kasabach-Merritt phenomenon arising from kaposiform hemangioendothelioma by sirolimus. J Pediatr Hematol Oncol. 37(1):72-3, 2015
                                                          16. Adams DM et al: Other vascular tumors. Semin Pediatr Surg. 23(4):173-7, 2014
                                                          17. Croteau SE et al: Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr. 162(1):142-7, 2013
                                                          18. Drolet BA et al: Consensus-derived practice standards plan for complicated kaposiform hemangioendothelioma. J Pediatr. 163(1):285-91, 2013
                                                          19. Fernández Y et al: Kaposiform hemangioendothelioma. Eur J Intern Med. 20(2):106-13, 2009
                                                          20. Navarro OM et al: Pediatric soft-tissue tumors and pseudotumors: MR imaging features with pathologic correlation: part 1. Imaging approach, pseudotumors, vascular lesions, and adipocytic tumors. Radiographics. 29(3):887-906, 2009
                                                          21. Enjolras O et al: Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol. 42(2 Pt 1):225-35, 2000
                                                          22. Beaubien ER et al: Kaposiform hemangioendothelioma: a locally aggressive vascular tumor. J Am Acad Dermatol. 38(5 Pt 2):799-802, 1998
                                                          23. Enjolras O et al: Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas. J Pediatr. 130(4):631-40, 1997
                                                          24. Zukerberg LR et al: Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol. 17(4):321-8, 1993