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Kaposiform Lymphangiomatosis
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Kaposiform lymphangiomatosis (KLA)
            • Definitions

              • Uncommon but distinct subtype of generalized lymphatic anomaly (GLA) with poor prognosis
                • Features of both neoplasia & malformation
              • Predominantly affects thorax with progressive respiratory symptoms & hemorrhages

            IMAGING

            • General Features

              DIFFERENTIAL DIAGNOSIS

                PATHOLOGY

                • Microscopic Features

                  CLINICAL ISSUES

                  • Presentation

                    • Demographics

                      • Natural History & Prognosis

                        • Treatment

                          Selected References

                          1. Iacobas I et al: Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies-expert opinion consensus. Pediatr Blood Cancer. e28036, 2019
                          2. Ji Y et al: Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences. Orphanet J Rare Dis. 14(1):165, 2019
                          3. Kato H et al: Chest imaging in generalized lymphatic anomaly and kaposiform lymphangiomatosis. Pediatr Int. 60(7):667-8, 2018
                          4. Kato H et al: MR imaging findings of vertebral involvement in Gorham-Stout disease, generalized lymphatic anomaly, and kaposiform lymphangiomatosis. Jpn J Radiol. 35(10):606-12, 2017
                          5. Goyal P et al: Imaging features of kaposiform lymphangiomatosis. Pediatr Radiol. 46(9):1282-90, 2016
                          6. Ozeki M et al: Clinical features and prognosis of generalized lymphatic anomaly, kaposiform lymphangiomatosis, and Gorham-Stout disease. Pediatr Blood Cancer. 63(5):832-8, 2016
                          7. Fernandes VM et al: Kaposiform lymphangiomatosis: unifying features of a heterogeneous disorder. Pediatr Blood Cancer. 62(5):901-4, 2015
                          8. Wang Z et al: Successful treatment of kaposiform lymphangiomatosis with sirolimus. Pediatr Blood Cancer. 62(7):1291-3, 2015
                          9. Croteau SE et al: Kaposiform lymphangiomatosis: a distinct aggressive lymphatic anomaly. J Pediatr. 164(2):383-8, 2014
                          10. Safi F et al: Kaposiform lymphangiomatosis, a newly characterized vascular anomaly presenting with hemoptysis in an adult woman. Ann Am Thorac Soc. 11(1):92-5, 2014
                          11. Trenor CC 3rd et al: Complex lymphatic anomalies. Semin Pediatr Surg. 23(4):186-90, 2014
                          Related Anatomy
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                          Related Differential Diagnoses
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                          References
                          Tables

                          Tables

                          KEY FACTS

                          • Terminology

                            • Imaging

                              • Top Differential Diagnoses

                                • Pathology

                                  • Clinical Issues

                                    TERMINOLOGY

                                    • Abbreviations

                                      • Kaposiform lymphangiomatosis (KLA)
                                    • Definitions

                                      • Uncommon but distinct subtype of generalized lymphatic anomaly (GLA) with poor prognosis
                                        • Features of both neoplasia & malformation
                                      • Predominantly affects thorax with progressive respiratory symptoms & hemorrhages

                                    IMAGING

                                    • General Features

                                      DIFFERENTIAL DIAGNOSIS

                                        PATHOLOGY

                                        • Microscopic Features

                                          CLINICAL ISSUES

                                          • Presentation

                                            • Demographics

                                              • Natural History & Prognosis

                                                • Treatment

                                                  Selected References

                                                  1. Iacobas I et al: Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies-expert opinion consensus. Pediatr Blood Cancer. e28036, 2019
                                                  2. Ji Y et al: Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences. Orphanet J Rare Dis. 14(1):165, 2019
                                                  3. Kato H et al: Chest imaging in generalized lymphatic anomaly and kaposiform lymphangiomatosis. Pediatr Int. 60(7):667-8, 2018
                                                  4. Kato H et al: MR imaging findings of vertebral involvement in Gorham-Stout disease, generalized lymphatic anomaly, and kaposiform lymphangiomatosis. Jpn J Radiol. 35(10):606-12, 2017
                                                  5. Goyal P et al: Imaging features of kaposiform lymphangiomatosis. Pediatr Radiol. 46(9):1282-90, 2016
                                                  6. Ozeki M et al: Clinical features and prognosis of generalized lymphatic anomaly, kaposiform lymphangiomatosis, and Gorham-Stout disease. Pediatr Blood Cancer. 63(5):832-8, 2016
                                                  7. Fernandes VM et al: Kaposiform lymphangiomatosis: unifying features of a heterogeneous disorder. Pediatr Blood Cancer. 62(5):901-4, 2015
                                                  8. Wang Z et al: Successful treatment of kaposiform lymphangiomatosis with sirolimus. Pediatr Blood Cancer. 62(7):1291-3, 2015
                                                  9. Croteau SE et al: Kaposiform lymphangiomatosis: a distinct aggressive lymphatic anomaly. J Pediatr. 164(2):383-8, 2014
                                                  10. Safi F et al: Kaposiform lymphangiomatosis, a newly characterized vascular anomaly presenting with hemoptysis in an adult woman. Ann Am Thorac Soc. 11(1):92-5, 2014
                                                  11. Trenor CC 3rd et al: Complex lymphatic anomalies. Semin Pediatr Surg. 23(4):186-90, 2014