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
Iacobas I et al: Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies-expert opinion consensus. Pediatr Blood Cancer. e28036, 2019
Ji Y et al: Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences. Orphanet J Rare Dis. 14(1):165, 2019
Kato H et al: Chest imaging in generalized lymphatic anomaly and kaposiform lymphangiomatosis. Pediatr Int. 60(7):667-8, 2018
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
Goyal P et al: Imaging features of kaposiform lymphangiomatosis. Pediatr Radiol. 46(9):1282-90, 2016
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
Fernandes VM et al: Kaposiform lymphangiomatosis: unifying features of a heterogeneous disorder. Pediatr Blood Cancer. 62(5):901-4, 2015
Wang Z et al: Successful treatment of kaposiform lymphangiomatosis with sirolimus. Pediatr Blood Cancer. 62(7):1291-3, 2015
Croteau SE et al: Kaposiform lymphangiomatosis: a distinct aggressive lymphatic anomaly. J Pediatr. 164(2):383-8, 2014
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
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
Iacobas I et al: Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies-expert opinion consensus. Pediatr Blood Cancer. e28036, 2019
Ji Y et al: Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences. Orphanet J Rare Dis. 14(1):165, 2019
Kato H et al: Chest imaging in generalized lymphatic anomaly and kaposiform lymphangiomatosis. Pediatr Int. 60(7):667-8, 2018
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
Goyal P et al: Imaging features of kaposiform lymphangiomatosis. Pediatr Radiol. 46(9):1282-90, 2016
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
Fernandes VM et al: Kaposiform lymphangiomatosis: unifying features of a heterogeneous disorder. Pediatr Blood Cancer. 62(5):901-4, 2015
Wang Z et al: Successful treatment of kaposiform lymphangiomatosis with sirolimus. Pediatr Blood Cancer. 62(7):1291-3, 2015
Croteau SE et al: Kaposiform lymphangiomatosis: a distinct aggressive lymphatic anomaly. J Pediatr. 164(2):383-8, 2014
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
Trenor CC 3rd et al: Complex lymphatic anomalies. Semin Pediatr Surg. 23(4):186-90, 2014
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