Clinical analysis of a family with familial hemangioblastoma
10.3760/cma.j.issn.1671-8925.2011.12.019
- VernacularTitle:家族性血管网状细胞瘤临床分析(附一家系报告)
- Author:
Ye-Shuai HU
1
;
Jun YANG
;
Shun-Chang MA
;
Jian-Fa QI
;
Ji-Jun LIANG
;
Zhi-Qiang LI
;
En-Hao CUN
;
Yong-Kai CHANG
;
Su-Hua CHEN
Author Information
1. 首都医科大学附属北京复兴医院
- Keywords:
Hemangioblastoma,familial;
VOH Hippel-Lindau;
Genetic disease
- From:
Chinese Journal of Neuromedicine
2011;10(12):1270-1273
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical and imaging features of a family(3 patients)with familial hemangioblastoma,and their diagnosis and prognosis.Methods The detailed data about clinical and imaging features of all patients diagnosed as familial hemangioblastoma,admitted to our hospital from October 2004 to May 2010,were analyzed,and the lesions of other regions,besides the tumor lesion,were observed.Results No lesions of other regions were noted in these 3 patients.Cranial MRI showed that 2 had cystic and solid tumor and 1 had solid tumor;,total removal was performed on these patients under microscope; regular follow-up was given and no recurrence was noted.Conclusion Familiar hemangioblastoma is serious hereditary disease; and MRI is the most important detective method; microsurgical operation is the most important therapy.Early diagnosis and treatment should be given to the patients with familiar hemangioblastoma due to its high recunence rate,having difficulty in operation and its trend to combining with other lesions of the other parts.
