摘要
目的探讨家族性血管母细胞瘤（ＨＢ）遗传学特征及其临床病理表现与非家族性ＨＢ有无差异。方法对５７例ＨＢ进行家系调查，长期随访及临床病理资料分析。结果本组１１例（１９．３％）为家族性发病，呈常染色体显性遗传。与非家族性ＨＢ比较，家族性ＨＢ术前病程短，肿瘤体积小，常呈多灶性，复发与再复发率高（Ｐ＜０．０５～０．０１）。性别比例、发病年龄、复发与再复发间隔时间及大体与组织学表现，两组间无显著性差异（Ｐ＞０．０５）。结论家族性与非家族性ＨＢ的主要差异为：术前病程、肿瘤体积、病灶多少及复发与再复发率的不同，对家族性病例术中必须注意全部切除所有病灶，并对其家庭成员进行系统检查。
bjective To approach heredity of familial hemangioblastomas(HB) and compare the clinicopathological appearance between familial HBs and nonfamilial ones. Methods A familial investigation,the long-term postoperative follow up and clinicopathological analysis of fifty-seven HBs were made. Results Eleven(19.3%) of them were familial occurrence, appeared autosomal dominant inheritance. The preoperative course of familial HBs was shorter, their size was smaller than nonfamilial cases( P <0.05-0.01). The familial HBs were often multicentral and had higher rate of recurrence and re-recurrence than nonfamilial HBs( P <0.05). However there was not significant difference in sex, onset age, interval of recurrence and re-recurrence, microscopic and histological appearance between the two groups( P >0.05). Conclusions The major distinctions between the familial and nonfamilial HBs are the differences in the preoperative course, tumor size, number of the focuses, recurrence and re-recurrence rates. To remove all focuses in the operation and to systematically examine all the family members are necessary in the familial cases.
出处
《中国神经精神疾病杂志》
CAS
CSCD
北大核心
1996年第6期334-337,共4页
Chinese Journal of Nervous and Mental Diseases
关键词
血管母细胞瘤
血管肿瘤
家族性
病理学
Hemangioblastoma Familial Inheritance Pathology Clinical analysis
