Clinical features, diagnosis and treatment of 2 pedigrees with familial hemangioblastoma
10.3760/cma.j.issn.1671-8925.2012.05.020
- VernacularTitle:两家系家族性颅内血管网状细胞瘤的临床特征及诊疗分析
- Author:
Lin LEI
1
;
Ya-Ping HUANG
;
Qing-Ru ZHU
Author Information
1. 湖北省襄阳市中心医院
- Keywords:
Hemangioblastoma;
Von Hippel-Lindau;
Clinical feature
- From:
Chinese Journal of Neuromedicine
2012;11(5):519-521
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical features,diagnosis and treatment of 2 pedigrees (5 patients) with familial hemangioblastoma. Methods The detailed clinical and imaging data of 2 pedigrees (5 patients) with familial hemangioblastoma,admitted to our hospital from September 2005 to May 2010, were retrospectively analyzed; their diagnosis and treatment were concluded. Results Among the 3 patients of the first pedigree,2 patients displayed cystic and solid tumor and 1 cystic tumor under cranial MRI; both 2 patients of the second pedigree displayed cystic solids.All the 5 patients had mutations and could be diagnosed as having yon Hippel-Lindau (VHL).No patients were combined with other parts of the lesion; after microscopic total/sub-total resection, no patients existed postoperative occurrence and no patient died. Conclusion MRI is the most important detective method in the diagnosis of patients with familiar hemangioblastomas; microsurgery is still the most important therapy method to familiar hemangioblastomas.
