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. 2003 Nov 1;63(21):7051-5.

Developmental arrest of angioblastic lineage initiates tumorigenesis in von Hippel-Lindau disease

Alexander O Vortmeyer  1 , 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
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  • PMID: 14612494

Developmental arrest of angioblastic lineage initiates tumorigenesis in von Hippel-Lindau disease

Alexander O Vortmeyer et al. Cancer Res. .

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.

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