von Hippel-Lindau 病相关性脊髓血管母细胞瘤切除术后的功能预后。

Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease.

机构信息

Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.

出版信息

J Neurosurg Spine. 2010 Mar;12(3):233-42. doi: 10.3171/2009.10.SPINE09592.

DOI:10.3171/2009.10.SPINE09592

PMID:20192620

Abstract

OBJECT

Spinal cord hemangioblastomas are a common protean manifestation of von Hippel-Lindau (VHL) disease and can be associated with significant morbidity. To better define expected outcome and optimal management of these tumors in the context of this neoplasia syndrome, the authors analyzed the findings from patients with VHL disease who underwent resection of spinal cord hemangioblastomas.

METHODS

Consecutive patients with VHL disease who underwent surgery for spinal cord hemangioblastomas with > 6 months follow-up were included in the study. Serial clinical examinations, functional scores, imaging findings, and operative records were analyzed.

RESULTS

One hundred eight patients (57 male, 51 female) underwent 156 operations for resection of 218 spinal cord hemangioblastomas. One hundred forty-six operations (94%) were performed for symptom-producing tumors. The most common presenting symptoms included hypesthesia (64% of resections), hyperreflexia (57%), dysesthesia (43%), and weakness (36%). Mean follow-up was 7.0 +/- 5.0 years (range 0.5-20.9 years). Complete resection was achieved for 217 tumors (99.5%). At 6-months follow-up, patients were stable or improved after 149 operations (96%) and worse after 7 operations (4%). Ventral tumors (OR 15.66, 95% CI 2.54-96.45; p = 0.003) or completely intramedullary tumors (OR 10.74, 95% CI 2.07-55.66; p = 0.005) were associated with an increased risk of postoperative worsening. The proportion of patients remaining functionally stable at 2, 5, 10, and 15 years' follow-up was 93, 86, 78, and 78%. Long-term functional decline was caused by extensive VHL-associated CNS disease (6 patients), VHL-associated visceral disease (1 patient), or non-VHL disease (2 patients).

CONCLUSIONS

Resection of symptomatic spinal cord hemangioblastomas is a safe and effective means of preserving neurological function in patients with VHL disease. Tumor location (ventral or completely intramedullary) can be used to assess functional risk associated with surgery. Long-term decline in neurological function is usually caused by VHL-associated disease progression.

摘要

目的

脊髓血管母细胞瘤是 von Hippel-Lindau（VHL）病的常见多变表现形式，可导致严重的发病率。为了更好地定义 VHL 病患者脊髓血管母细胞瘤在这种肿瘤综合征背景下的预期结果和最佳管理，作者分析了接受脊髓血管母细胞瘤切除术的 VHL 病患者的发现。

方法

纳入了接受脊髓血管母细胞瘤切除术且随访时间超过 6 个月的连续 VHL 病患者。分析了连续的临床检查、功能评分、影像学发现和手术记录。

结果

108 例患者（57 例男性，51 例女性）接受了 156 次手术切除 218 个脊髓血管母细胞瘤。146 次手术（94%）是为有症状的肿瘤进行的。最常见的首发症状包括感觉迟钝（64%的切除术）、反射亢进（57%）、感觉异常（43%）和无力（36%）。平均随访时间为 7.0+/-5.0 年（范围 0.5-20.9 年）。217 个肿瘤（99.5%）达到完全切除。在 6 个月的随访中，149 次手术（96%）后患者稳定或改善，7 次手术（4%）后恶化。腹侧肿瘤（OR 15.66，95%CI 2.54-96.45；p=0.003）或完全髓内肿瘤（OR 10.74，95%CI 2.07-55.66；p=0.005）与术后恶化风险增加相关。2、5、10 和 15 年随访时，功能稳定的患者比例分别为 93%、86%、78%和 78%。长期的功能下降是由广泛的 VHL 相关中枢神经系统疾病（6 例）、VHL 相关内脏疾病（1 例）或非 VHL 疾病（2 例）引起的。