Proteomic analysis of hemangioblastoma cyst fluid.
OBJECTIVE
Hemangioblastomas are benign CNS tumors that occur sporadically or in patients with von Hippel-Lindau (VHL) disease. These tumors are characteristically associated with formation of intra- or peritumoral cysts. Hemangioblastoma cyst formation is a major cause of morbidity and mortality with these tumors. While peritumoral cysts have been suggested to result from vascular leakage, the mechanism of intratumoral cyst formation is not understood.


METHODS
To elucidate the origin of intratumoral hemangioblastoma cyst fluid, we characterized its biochemical composition by two-dimensional (2D) proteomic profiling followed by sequencing of several proteins. The proteomic pattern of intratumoral cyst fluid was furthermore compared to the proteomic pattern of serum, hemangioblastoma tumor tissue, and hemangioblastoma peritumoral cyst fluid.


RESULTS
We show that proteomic patterns of intra- and peritumoral cyst fluid are identical Both are highly similar to serum and not to tumor.


CONCLUSIONS
Intratumoral hemangioblastoma cyst fluid originates from serum. Cyst formation associated with hemangioblastoma, whether peri- or intratumoral, is a consequence of vascular leakage. Anti-VEGF therapy may effectively control hemangioblastoma cyst formation.
Gläsker
Sven
S
Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA.
Vortmeyer
Alexander O
AO
Lonser
Russell R
RR
Lubensky
Irina A
IA
Okamoto
Hiroaki
H
Xia
John B
JB
Li
Jie
J
Milne
Erin
E
Kowalak
Jeffrey A
JA
Oldfield
Edward H
EH
Zhuang
Zhengping
Z
D014408:Biomarkers, Tumor / Q000378:metabolism; D002528:Cerebellar Neoplasms / Q000378:metabolism* / Q000473:pathology; D002853:Chromatography, Liquid; D020420:Cyst Fluid / Q000378:metabolism*; D015180:Electrophoresis, Gel, Two-Dimensional; D018325:Hemangioblastoma / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D013058:Mass Spectrometry; D020543:Proteome / Q000032:analysis*
D014408:Biomarkers, Tumor; D020543:Proteome