Lymphatic Malformations

  • Occur in-utero
  • Somatic mutation in PIK3C
  • Types
    • Macrocystic LM - bigger cavities - more amenable to sclerotherapy
    • Microcystic LM - smaller cavities - Lymphangioma circunscriptum on the tongue
    • Mixed LM
  • Risk
    • Recurrent severe infections and lymphatic leak
  • Exames complementares de diagnóstico
    • Estudo genético: EML4:ALK4, ROS1 fusion
  • Treatment:
    • Sirolimus
    • Sclerotherapy
    • Surgical debulking
    • CO2 laser for surface of microcystic LM