Clinical features of patients bearing central nervous system hemangioblastoma in von Hippel-Lindau disease
Clinical features of patients bearing central nervous system hemangioblastoma in von Hippel-Lindau disease
复制标题
冯·希佩尔-林道病中枢神经系统血管母细胞瘤患者的临床特征
作者：
H. Kanno;J. Kuratsu;R. Nishikawa;K. Mishima;A. Natsume;T. Wakabayashi;K. Houkin;S. Terasaka;T. Shuin
中枢神经系统（CNS）血管母细胞瘤（HB）是von Hippel-Lindau病（VHL）最常见的临床表现之一，但对VHL中CNS HB的临床特征缺乏大规模的研究。方法在问卷调查结果的基础上，收集111例VHL患者的CNS HB资料。结果111例VHL患者中CNS HB的总数为1000例，占VHL患者的100%，占VHL患者的10%。264例（男59例，女52例），分布部位：小脑65.4%，脑干9.9%，脊髓23.9%，垂体1.0%。百分之一随访时间0.6 ~ 39.2年，平均12.5年。患有脑干或脊髓HB的患者发生另一种HB的频率也明显高于患有小脑HB的患者（P < 0.05）。CNS HB的平均发病年龄为29.1岁，单HB患者的平均发病年龄（34.4岁）明显大于多HB患者（25.7岁）。40岁以下的多乙肝患者比单乙肝患者更占优势。脑干HB在30岁以下患者中的分布率明显小于29岁以上患者。尽管ECOG PS评分随着手术次数的增加而沿着增加，但发病年龄随着手术次数的增加而降低。20岁以下患者的平均ECOG PS评分显著小于19岁以上患者。结论CNS HB发病年龄在40岁以下，且CNS HB位于脑干或脊髓时，可预测其多发概率。由于发病年龄在20岁以下的患者保持了较高的体能状态，因此CNS HB的早期检测非常重要。此外，由于多次操作会恶化性能状态，因此应减少操作次数。
BackgroundCentral nervous system (CNS) hemangioblastoma (HB) is one of the most common manifestations in von Hippel-Lindau disease (VHL), but large-scale studies on clinical features of CNS HB in VHL are scarce.MethodsOn the basis of the results of a questionnaire, we collected data of VHL patients with CNS HB.ResultsThe total number of CNS HBs in 111 VHL patients (male 59, female 52) was 264 with the following distributions: cerebellar, 65.4 %; brainstem, 9.9 %; spinal cord, 23.9 %; and pituitary, 1. 1 %. The follow-up period was 0.6 to 39.2 years, with the mean 12.5 years. Patients bearing brainstem or spinal cord HB also had another HB significantly more frequently than those bearing cerebellar HBs (P < 0.05). The mean onset age of CNS HB was 29.1 years, and that of patients bearing a single HB (mean 34.4 years) was significantly greater than that of multiple HBs (mean 25.7 years). Patients with multiple HBs under 40 years are more dominant than those with a single HB. The distribution rate of brainstem HB is significantly smaller in patients below 30 years than patients above 29 years. Although ECOG PS score increased along with number of operations, the onset age decreased with increasing number of operations. The mean ECOG PS score of patients below 20 years is significantly smaller than patients above 19 years.ConclusionsWhen the onset age of CNS HB is under 40 years, and CNS HB is located at the brainstem or spinal cord HB, the probability of multiple occurrence can be predicted. Since patients with an onset age under 20 years old preserve a high performance status, early detection of CNS HB would be important. In addition, since a multiple operations aggravate performance status, number of operations should be reduced.
影响因子：
4.1
作者：
Wanebo, JE;Lonser, RR;Oldfield, EH
通讯作者：
Oldfield, EH
影响因子：
4.1
作者：
Ammerman, Joshua M.;Lonser, Russell R.;Oldfield, Edward H.
通讯作者：
Oldfield, Edward H.