Leonov M A, Makhmudov U B, Shimanskiĭ V N
Zh Vopr Neirokhir Im N N Burdenko. 2003 Oct-Dec(4):39-44.
Hemangioblastomas of the central nervous system are benign, richly blood-supplied tumors that may be encountered in its any part, more commonly in the cerebellar hemispheres. Supratentorially, they occur rarely. In the literature there are reports on less than 100 cases of tumors at this site. The histogenesis of hemangioblastomas has not been clearly ascertained so far. Magnetic resonance imaging is the only valid diagnostic study of hemangioblastomas. Hemangioblastomas may be presented as sporadic forms and a manifestation of the Hippel-Lindau syndrome. In the latter case, hemangioblastomas are associated with angiomas of the retina and other viscera. Surgery of hemangioblastomas is the basic treatment. Radiosurgery is the method of choice for patients with Hippel-Lindau disease. The main cause of postoperative death is bleeding to the brain stem and the remaining portions of the tumor. Endovascular embolization of the vessels supplying blood to the tumor may be used to diminish postoperative bleeding. Recurrences are mainly associated with incomplete surgical tumor removal. Chemotherapy is not indicated in hemangioblastomas. Radiation therapy may be used after partial tumor removal.
中枢神经系统血管母细胞瘤是良性、血供丰富的肿瘤,可发生于中枢神经系统的任何部位,更常见于小脑半球。幕上部位较少见。文献报道该部位肿瘤病例不足100例。目前血管母细胞瘤的组织发生尚未明确。磁共振成像(MRI)是血管母细胞瘤唯一有效的诊断检查。血管母细胞瘤可表现为散发形式及希佩尔-林道综合征(VHL综合征)的一种表现。在后一种情况下,血管母细胞瘤与视网膜及其他内脏的血管瘤相关。血管母细胞瘤的手术是基本治疗方法。立体定向放射外科手术是VHL病患者的首选治疗方法。术后死亡的主要原因是脑干出血及肿瘤残留部分出血。对肿瘤供血血管进行血管内栓塞可减少术后出血。复发主要与手术未能完全切除肿瘤有关。血管母细胞瘤不适用化疗。部分切除肿瘤后可采用放射治疗。