Hemangioblastomas share protein expression with embryonal hemangioblast progenitor cell

Sven Gläsker, Jie Li, John B. Xia, Hiroaki Okamoto, Weifen Zeng, Russell R. Lonser, Zhengping Zhuang, Edward H. Oldfield, Alexander O. Vortmeyer

Research output: Contribution to journalArticlepeer-review

69 Scopus citations

Abstract

Hemangioblastomas are central nervous system (CNS) tumors of unknown histogenesis, which can occur sporadically or in von Hippel-Lindau disease. Hemangioblastomas are composed of neoplastic "stromal" cells of unknown origin, accompanied by intensive reactive angiogenesis. Failure to specify the cytologic origin of the stromal cell has precluded the development of nonsurgical therapies and limits understanding of its basic biology. We report that the stromal cells express proteins (Scl, brachyury, Csf-1R, Gata-1, Flk-1, and Tie-2) that characterize embryonic progenitor cells with hemangioblastic differentiation potential and conclude that embryonic progenitors with hemangioblast potential represent a possible cytologic equivalent of the stromal cell. We also identified a new autocrine/ paracrine stimulatory loop between the receptor Tie-2 and the hypoxia-inducible factor target Ang-1, which, combined with previous observations, suggests that a variety of autocrine loops may be initiated in hemangioblastomas, depending on the differentiation status of the tumor cells and the extent of HIF downstream activation. Finally, the consistent identification of Scl in the stromal cells may help explain the unique and characteristic topographical distribution of hemangioblastomas within the CNS.

Original languageEnglish (US)
Pages (from-to)4167-4172
Number of pages6
JournalCancer Research
Volume66
Issue number8
DOIs
StatePublished - Apr 15 2006

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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