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Difference in CXCR4 expression between sporadic and VHL-related hemangioblastoma

Content Provider	Springer Nature Link
Author	Kruizinga, Roeliene C. Marion, Denise M. S. Dunnen, Wilfred F. A. Groot, Jan C. Hoving, Eelco W. Oosting, Sjoukje F. Timmer Bosscha, Hetty Derks, Rosalie P. H. Cornelissen, Chantal Luijt, Rob B. Links, Thera P. Vries, Elisabeth G. E. Walenkamp, Annemiek M. E.
Copyright Year	2016
Abstract	Central nervous system hemangioblastomas occur sporadically and in patients with von Hippel–Lindau (VHL) disease due to a VHL germline mutation. This mutation leads to enhanced transcription of chemokine receptor 4 (CXCR4), its ligand (CXCL12) and vascular endothelial growth factor A (VEGFA). We aimed to determine in VHL-related and sporadic hemangioblastomas CXCR4, CXCL12, and VEGFA protein expression and to correlate this to hemangioblastoma size and expression in normal surrounding tissue. 27 patients with a hemangioblastoma were included for analysis of immunohistochemistry of tissue, MRI and DNA. Hemangioblastomas overexpress CXCR4, CXCL12, and VEGFA compared to normal surrounding tissue. In sporadic hemangioblastomas the mean percentage of CXCR4 positive hemangioblastoma cells was 16 %, SD 8.4, in VHL-related hemangioblastomas 8 %, SD 4.4 (P = 0.002). There was no relation between preoperative tumor size and CXCR4 or CXCL12 expression. Compared to normal surrounding tissue CXCR4, CXCL12, and VEGFA were overexpressed in hemangioblastomas. Most interestingly, sporadic hemangioblastomas overexpress CXCR4 compared to VHL-related hemangioblastoma.
Starting Page	607
Ending Page	616
Page Count	10
File Format	PDF
ISSN	13899600
Journal	Familial Cancer
Volume Number	15
Issue Number	4
e-ISSN	15737292
Language	English
Publisher	Springer Netherlands
Publisher Date	2016-02-26
Publisher Place	Dordrecht
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	CXCR4 CXCL12 Hemangioblastomas VEGF VHL Cancer Research Human Genetics Epidemiology Biomedicine general
Content Type	Text
Resource Type	Article
Subject	Genetics Cancer Research Genetics (clinical) Oncology

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