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10.1093/neuonc/nou206.32

http://scihub22266oqcxt.onion/10.1093/neuonc/nou206.32
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suck abstract from ncbi


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pmidC4144498      Neuro+Oncol 2014 ; 16 (Suppl 3): iii9
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  • VASCULOGENESIS IN VON HIPPEL-LINDAU DISEASE ASSOCIATED TUMORS #MMPMIDC4144498
  • Lonser RR; Frerich J; Huntoon K; Yang C; Merrill M; Abdullaev Z; Pack S; Shively S; Stamp G; Zhuang Z
  • Neuro Oncol 2014[Jul]; 16 (Suppl 3): iii9 PMIDC4144498show ga
  • BACKGROUND: Emerging data indicate that von Hippel-Lindau disease (VHL) associated tumors arise from embryologic hemangioblasts that can form vessels (endothelial cells) and blood cells. Nevertheless, the origin of VHL-associated vasculature is not known. To determine the origin of VHL-associated tumor vasculature, we investigated the neoplastic vasculature from VHL patients. METHODS: Microdissected VHL-associated tumor (compared to control non-VHL tissues) vascular features were examined using immunohistochemical staining for CA9, HIF-2a, HIF-1a, CD31 and Factor VIIIa. Origin of tumor vascular elements (tumor versus non-tumor) was assessed by LOH and FISH analysis. Intratumoral vasculogenesis was assessed in vivo using the VHL-deficient UMRC6 renal carcinoma murine xenograft model. RESULTS: We demonstrate that isolated vascular structures and blood vessels within VHL-associated neoplasms (including hemangioblastomas, renal cell carcinoma and pancreatic tumors) are a result of tumor-derived vasculogenesis. Further, similar to hemangioblastomas, other VHL-associated neoplasms possess vascular tissue of tumor origin. Similarly, tumor-derived endothelial cells emerge within implanted VHL deficient UMRC6 renal cell carcinoma murine xenografts. CONCLUSIONS: These findings further establish the embryologic, developmentally arrested, hemangioblast as the tumor cell of origin for VHL-associated hemangioblastomas and indicate that it is also the progenitor cell for other VHL-associated tumors. SECONDARY CATEGORY: Neuropathology & Tumor Biomarkers.
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