title: HIF transcription factor expression and induction of hypoxic response genes in a retroperitoneal angiosarcoma.
abstract: Angiosarcoma is a rare and highly aggressive tumor of endothelial origin. The molecular mechanisms driving angiosarcoma growth have not been fully elucidated, although autocrine stimulation by vascular endothelial growth factor (VEGF) secretion may play a role in the pathogenesis of this tumor. We identified a patient with a very rare form of angiosarcoma arising from the retroperitoneum. Immunohistochemical analysis demonstrated widespread up-regulation of the hypoxic response pathway as a mechanism of enhanced VEGF expression. Disordered regulation of the hypoxic response pathway can result in the expression of factors such as VEGF and erythropoietin, which may promote autocrine tumor growth in angiosarcoma.
authors.affiliation: Division of Hematology/Oncology, Lineberger Comprehensive Cancer Center, University of North Carolina at Chapel Hill, 27599, USA. rathmell@med.unc.edu
authors.forename: W Kimryn
authors.initials: WK
authors.lastname: Rathmell
authors.forename: Geza
authors.initials: G
authors.lastname: Acs
authors.forename: M Celeste
authors.initials: MC
authors.lastname: Simon
authors.forename: David J
authors.initials: DJ
authors.lastname: Vaughn
mesh_terms: D051792:Basic Helix-Loop-Helix Proteins; D015972:Gene Expression Regulation, Neoplastic; D006394:Hemangiosarcoma / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D051795:Hypoxia-Inducible Factor 1, alpha Subunit; D008297:Male; D008875:Middle Aged; D012186:Retroperitoneal Neoplasms / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D015534:Trans-Activators / Q000096:biosynthesis* / Q000235:genetics; D014157:Transcription Factors / Q000096:biosynthesis* / Q000235:genetics; D000099162:Endothelial PAS Domain-Containing Protein 1
chemical_list: D051792:Basic Helix-Loop-Helix Proteins; D051795:Hypoxia-Inducible Factor 1, alpha Subunit; D015534:Trans-Activators; D014157:Transcription Factors; D000099162:Endothelial PAS Domain-Containing Protein 1; C497442:HIF1A protein, human