Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892-1414, USA.
Neuro Oncol. 2010 Jan;12(1):80-6. doi: 10.1093/neuonc/nop018. Epub 2009 Dec 23.
To determine the effectiveness of stereotactic radiosurgery (SRS) treatment to central nervous system (CNS) hemangioblastomas in von Hippel-Lindau disease (VHL), we analyzed long-term results in VHL patients treated with SRS. Patients were enrolled in a prospective VHL natural history study, undergoing SRS treatment of CNS hemangioblastomas. Treatment regimens, serial clinical evaluations, and longitudinal imaging data were analyzed. Twenty VHL patients (10 males and 10 females) underwent SRS treatment of 44 CNS hemangioblastomas (39 cerebellar and 5 brainstem). Mean (+/-SD) age at treatment was 37.5 +/- 12.0 years (range: 13-67). Mean follow-up was 8.5 +/- 3.2 years (range: 3.0-17.6 years). All patients were alive at last follow-up. Mean treated tumor volume was 0.5 +/- 0.7 cm(3) (range: 0.01-3.6 cm(3)). Mean prescription dose was 18.9 Gy (range: 12-24 Gy) at the tumor margin. Local control rate at 2, 5, 10, and 15 years after SRS treatment was 91%, 83%, 61%, and 51%, respectively. Univariate analysis did not identify variables associated (P > .05) with worse tumor control at last follow-up. Thirty-three percent of SRS-treated small (<1.0 cm diameter), asymptomatic tumors progressed over a long-term follow-up. There were no long-term adverse radiation effects. Although SRS treatment of hemangioblastomas in VHL has a low risk for adverse radiation effects, it is associated with diminishing control over a long-term follow-up. These results indicate that SRS should not be used to prophylactically treat asymptomatic tumors and should be reserved for the treatment of tumors that are not surgically resectable.
为了确定立体定向放射外科（SRS）治疗中枢神经系统（CNS）血管母细胞瘤在von Hippel-Lindau 病（VHL）中的效果，我们分析了接受 SRS 治疗的 VHL 患者的长期结果。患者参加了一项前瞻性的 VHL 自然史研究，接受了 CNS 血管母细胞瘤的 SRS 治疗。分析了治疗方案、连续临床评估和纵向成像数据。20 名 VHL 患者（男性 10 名，女性 10 名）接受了 44 个 CNS 血管母细胞瘤（39 个小脑和 5 个脑干）的 SRS 治疗。治疗时的平均（+/-SD）年龄为 37.5 +/- 12.0 岁（范围：13-67）。平均随访时间为 8.5 +/- 3.2 年（范围：3.0-17.6 年）。最后一次随访时所有患者均存活。平均治疗肿瘤体积为 0.5 +/- 0.7 cm(3)（范围：0.01-3.6 cm(3)）。肿瘤边缘的平均处方剂量为 18.9 Gy（范围：12-24 Gy）。SRS 治疗后 2、5、10 和 15 年的局部控制率分别为 91%、83%、61%和 51%。单因素分析未发现与最后随访时肿瘤控制较差相关的变量（P>.05）。33%的 SRS 治疗小（<1.0 cm 直径）、无症状肿瘤在长期随访中进展。无长期放射性不良反应。尽管 SRS 治疗 VHL 中的血管母细胞瘤的放射不良反应风险较低，但随着长期随访，其控制效果逐渐减弱。这些结果表明，SRS 不应用于预防性治疗无症状肿瘤，而应保留用于治疗无法手术切除的肿瘤。
