Endothelial cell tumors.

Historically, there has been much debate on the nature of infantile hemangiomas as either congenital malformations or benign neoplasms. Some vascular lesions that are present at birth and evidence no proliferative growth are considered to be congenital malformations; other post-natal vascular tumors pursue aggressive and possibly lethal clinical courses. The literature of the last two decades has been reviewed with a hope of clarifying the pathogenesis and underlying molecular lesions of this diverse set of lesions. Genetic investigations of two diseases associated with vascular tumors and abnormalities, von Hippel-Lindau disease, and Hereditary Hemorrhagic Telangiectasia have greatly added to our knowledge of vascular proliferation and provided a tantalizing clue to the pathogenesis of hemangioblastomas. Mutations have also been described in infantile hemangiomas. All of the entities considered, vascular neoplasms as well as malformations, have been examined for the expression of vascular growth factors, their receptors, and factors that appear to promote cell proliferation. Similarly, factors that either block or promote apoptosis have also been examined in various vascular lesions. These studies have in large confirmed our expectations about proliferating tumors that show upregulation of growth promoting factors and inhibition of those that promote apoptosis. In conclusion, although much has been learned about vascular physiology and the control of endothelial proliferation, and while understanding about the molecular pathogenesis of the two inherited diseases mentioned above is detailed but not yet complete, understanding of the pathogenesis of benign and malignant endothelial tumors remains vague. Copyright 2003 Wiley-Liss, Inc.