Multiple mouse model- and human sample-based analyses have revealed that an increase in EC activation and a local inhibition of ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type I repeats 13), an enzyme that cleaves and inactivates VWF multimers under physiological conditions [22], are responsible for the occurrence of VWF fibers in the tumor microenvironment. This evidence concerns the gene ADAMTS13 and neoplasm.