Background: Angiopoietin-2 (Ang-2) is increased in the blood of patients with kaposiform lymphangiomatosis (KLA) and kaposiform hemangioendothelioma (KHE). While the genetic causes of KHE are not clear, a somatic activating NRAS mutation has been found in the lesions of KLA patients.
Procedure: Our study tested the hypothesis that the NRAS mutation drives elevated Ang-2 expression in endothelial cells.