Developmental Arrest of Angioblastic Lineage Initiates Tumorigenesis in von Hippel-Lindau Disease

Alexander O. Vortmeyer, Stephan Frank, Seon Yong Jeong, Kristy Yuan, Barbara Ikejiri, Youn Soo Lee, Deb Bhowmick, Russell R. Lonser, Reginald Smith, Griffin Rodgers, Edward H. Oldfield, Zhengping Zhuang

Research output: Contribution to journalArticle

82 Scopus citations

Abstract

The nature of the cell responsible for von Hippel-Lindau (VHL) disease-associated tumor formation has been controversial for decades. We demonstrate that VHL disease-associated central nervous system tumors are composed of developmentally arrested angioblasts that coexpress erythropoietin (Epo) and Epo receptor. The angioblasts are capable of differentiating into RBCs via formation of blood islands with extramedullary hematopoiesis. Because of VHL deficiency, Epo receptor-expressing, developmentally arrested angioblasts simultaneously coexpress Epo, which may represent a crucial pathogenetic step in tumor formation.

Original languageEnglish (US)
Pages (from-to)7051-7055
Number of pages5
JournalCancer Research
Volume63
Issue number21
StatePublished - Nov 1 2003
Externally publishedYes

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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    Vortmeyer, A. O., Frank, S., Jeong, S. Y., Yuan, K., Ikejiri, B., Lee, Y. S., Bhowmick, D., Lonser, R. R., Smith, R., Rodgers, G., Oldfield, E. H., & Zhuang, Z. (2003). Developmental Arrest of Angioblastic Lineage Initiates Tumorigenesis in von Hippel-Lindau Disease. Cancer Research, 63(21), 7051-7055.