Sacko O, Bouillot-Eimer S, Sesay M, Uro-Coste E, Roux F-E, Loiseau H
Department of Neurosurgery, Pellegrin University Hospital, 33076 Bordeaux, France.
Neurochirurgie. 2010 Oct;56(5):382-5. doi: 10.1016/j.neuchi.2009.11.006. Epub 2009 Dec 31.
A third case of corpus callosum hemangioblastoma (HB) is presented. With no preoperative embolization, surgery was uneventful and the postoperative course was excellent. Based on the literature, we attempted to clarify the histogenesis of HB and to explain why they are exceptional in the supratentorial region in contrast to the posterior cranial fossa. The VHL gene is expressed particularly in Purkinje cells of the cerebellum, but this expression is also possible in supratentorial structures. Its mutation leads to developmental arrest of angioblasts that become potentially neoplastic cells. These CD133-positive pluripotent neoplastic angioblasts, similar to stem cells, may be immature HB in the brain. They also express VEGF, coexpress Epo/EpoR, and are capable of differentiation into primitive vascular structures. This coexpression may not only mediate developmental stagnation, but may also induce HB proliferation. Therefore, HB tumorigenesis may be initiated during embryogenesis and may originate from angiomesenchyma because of the expression of three cell types (stromal cells, pericytes, and endothelial cells) in vimentin. Their capacity for proliferation and differentiation in HB depends on the microenvironment.
本文报告了第三例胼胝体血管母细胞瘤（HB）。未进行术前栓塞，手术顺利，术后恢复良好。基于文献，我们试图阐明HB的组织发生，并解释为何与后颅窝相比，它们在幕上区域较为罕见。VHL基因特别在小脑的浦肯野细胞中表达，但在幕上结构中也可能表达。其突变导致成血管细胞发育停滞，这些成血管细胞可能成为肿瘤细胞。这些CD133阳性的多能肿瘤性成血管细胞类似于干细胞，可能是脑内未成熟的HB。它们还表达VEGF，共表达Epo/EpoR，并能够分化为原始血管结构。这种共表达不仅可能介导发育停滞，还可能诱导HB增殖。因此，HB的肿瘤发生可能在胚胎发育过程中启动，并且可能由于波形蛋白中三种细胞类型（基质细胞、周细胞和内皮细胞）的表达而起源于血管间充质。它们在HB中的增殖和分化能力取决于微环境。
