Gläsker Sven, Vortmeyer Alexander O, Lonser Russell R, Lubensky Irina A, Okamoto Hiroaki, Xia John B, Li Jie, Milne Erin, Kowalak Jeffrey A, Oldfield Edward H, Zhuang Zhengping
Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA.
Cancer Biol Ther. 2006 May;5(5):549-53. doi: 10.4161/cbt.5.5.2657. Epub 2006 May 6.
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.
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.
We show that proteomic patterns of intra- and peritumoral cyst fluid are identical Both are highly similar to serum and not to tumor.
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.
成血管细胞瘤是一种良性中枢神经系统肿瘤，可散发发生或见于患有冯·希佩尔-林道（VHL）病的患者。这些肿瘤的特征是与瘤内或瘤周囊肿的形成有关。成血管细胞瘤囊肿的形成是这些肿瘤发病和死亡的主要原因。虽然有人认为瘤周囊肿是血管渗漏所致，但瘤内囊肿形成的机制尚不清楚。
为了阐明成血管细胞瘤瘤内囊肿液的来源，我们通过二维（2D）蛋白质组分析对其生化组成进行了表征，随后对几种蛋白质进行了测序。此外，还将瘤内囊肿液的蛋白质组模式与血清、成血管细胞瘤肿瘤组织和成血管细胞瘤瘤周囊肿液的蛋白质组模式进行了比较。
我们发现瘤内和瘤周囊肿液的蛋白质组模式是相同的。两者都与血清高度相似，而与肿瘤不同。
成血管细胞瘤瘤内囊肿液源自血清。与成血管细胞瘤相关的囊肿形成，无论是瘤周还是瘤内，都是血管渗漏的结果。抗血管内皮生长因子（VEGF）治疗可能有效控制成血管细胞瘤囊肿的形成。
