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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. Blei F: Kaposiform hemangioendothelioma: therapeutic efficacy for an enigmatic diagnosis. Pediatr Blood Cancer. 62(4):551-2, 2015
                              2. 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
                              3. 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
                              4. Adams DM et al: Other vascular tumors. Semin Pediatr Surg. 23(4):173-7, 2014
                              5. Azma R et al: Multifocal kaposiform hemangioendothelioma of soft tissue with bilateral pulmonary involvement in an adolescent. Korean J Pediatr. 57(11):500-4, 2014
                              6. Calvo-Garcia MA et al: Imaging evaluation of fetal vascular anomalies. Pediatr Radiol. ePub, 2014
                              7. Nakaya T et al: Multifocal kaposiform hemangioendothelioma in multiple visceral organs: an autopsy of 9-day-old female baby. Hum Pathol. 45(8):1773-7, 2014
                              8. Croteau SE et al: Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr. 162(1):142-7, 2013
                              9. Drolet BA et al: Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma. J Pediatr. 163(1):285-91, 2013
                              10. Tamai N et al: Kaposiform hemangioendothelioma arising in the deltoid muscle without the Kasabach-Merritt phenomenon. Skeletal Radiol. 39(10):1043-6, 2010
                              11. Fernández Y et al: Kaposiform hemangioendothelioma. Eur J Intern Med. 20(2):106-13, 2009
                              12. 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
                              13. Mulliken JB et al: Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 13-2004. A newborn girl with a large cutaneous lesion, thrombocytopenia, and anemia. N Engl J Med. 350(17):1764-75, 2004
                              14. Enjolras O et al: Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol. 42(2 Pt 1):225-35, 2000
                              15. Beaubien ER et al: Kaposiform hemangioendothelioma: a locally aggressive vascular tumor. J Am Acad Dermatol. 38(5 Pt 2):799-802, 1998
                              16. Enjolras O et al: Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas. J Pediatr. 130(4):631-40, 1997
                              17. 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. Blei F: Kaposiform hemangioendothelioma: therapeutic efficacy for an enigmatic diagnosis. Pediatr Blood Cancer. 62(4):551-2, 2015
                                                          2. 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
                                                          3. 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
                                                          4. Adams DM et al: Other vascular tumors. Semin Pediatr Surg. 23(4):173-7, 2014
                                                          5. Azma R et al: Multifocal kaposiform hemangioendothelioma of soft tissue with bilateral pulmonary involvement in an adolescent. Korean J Pediatr. 57(11):500-4, 2014
                                                          6. Calvo-Garcia MA et al: Imaging evaluation of fetal vascular anomalies. Pediatr Radiol. ePub, 2014
                                                          7. Nakaya T et al: Multifocal kaposiform hemangioendothelioma in multiple visceral organs: an autopsy of 9-day-old female baby. Hum Pathol. 45(8):1773-7, 2014
                                                          8. Croteau SE et al: Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr. 162(1):142-7, 2013
                                                          9. Drolet BA et al: Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma. J Pediatr. 163(1):285-91, 2013
                                                          10. Tamai N et al: Kaposiform hemangioendothelioma arising in the deltoid muscle without the Kasabach-Merritt phenomenon. Skeletal Radiol. 39(10):1043-6, 2010
                                                          11. Fernández Y et al: Kaposiform hemangioendothelioma. Eur J Intern Med. 20(2):106-13, 2009
                                                          12. 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
                                                          13. Mulliken JB et al: Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 13-2004. A newborn girl with a large cutaneous lesion, thrombocytopenia, and anemia. N Engl J Med. 350(17):1764-75, 2004
                                                          14. Enjolras O et al: Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol. 42(2 Pt 1):225-35, 2000
                                                          15. Beaubien ER et al: Kaposiform hemangioendothelioma: a locally aggressive vascular tumor. J Am Acad Dermatol. 38(5 Pt 2):799-802, 1998
                                                          16. Enjolras O et al: Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas. J Pediatr. 130(4):631-40, 1997
                                                          17. 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