Interference of angiogenic growth factor signaling with junctional
proteins and vascular leakage in GBM. (a) In the normal brain
vasculature, paracrine Ang-1 signaling leads to the phosphorylation and
subsequent translocation of Tie2 to endothelial cell junctions (EC
junctions; upper right panel). VEGF receptor 2 (VEGFR2) is activated due
to low amounts of the activating ligand VEGF, but a stable vasculature
is maintained by concomitant Tie2 activation. In GBM, several mechanisms
lead to the induction of vascular leakage: (1) Ang-2 is secreted from
Weibel–Palade bodies and acts in an autocrine manner on Tie2, thereby
blocking the Ang1-induced Tie2 phosphorylation and vessel stabilization
(lower right panel). (2) Tie2 phosphorylation is further reduced by the
activity of VE-PTP that dephosphorylates Tie2 at EC junctions, thereby
promoting vascular permeability. VE-PTP expression is increased in
hypoxic endothelial cells and in neovascular endothelial cells in mice
with ischemic retinopathy suggesting increased expression in tumor vessels.76 (3) Local VEGF levels are increased leading to vascular
endothelial cadherin (VE-cadherin) phosphorylation and internalization
in an SRC kinase-dependent manner. Dexamethasone interferes with and
prevents vascular leakage by a yet unknown mechanism that may include
inhibition/downregulation of VEGF, whereas anti-VEGF treatment (BEV)
neutralizes excess VEGF, and VE-PTP inhibition (AKB-9778) reduces the
catalytic activity of VE-PTP to restore Tie2 activation to prevent
vascular leakage. Illustration: Visual Science
Communication. (b) Illustration of brain edema in GBM
patients. Upon DEX treatment (left), the T2 weighted cMRI signal shows
the anti-edema effect of DEX when compared with the DEX naive scenario
in a GBM patient with perifocal edema (encircled in blue) in the left
parietooccipital lobe. Upon BEV (anti-VEGF) therapy of another GBM
patient (right), a weighted cMRI axial section shows decreased T2 signal
enhancement demonstrating the anti-edema effect of BEV compared to
progressive disease and perifocal edema (encircled in blue) of the
corpus calosum before treatment.