The Diagnosis And Treatment Of Central Nervous System Hemangioblastoma

Objective:Hemangioblastoma are rare central nerveous system(CNS)tumor.The purpose of this study is to summarize its clinical and imaging features and to discuss the diagnosis and treatment.Meathods:We reviewed the history,presentation,imaging results,diagnoses and differential diagnoses of 104 patients with hemangioblastomas of CNS in the 2nd affiliated hospital to Zhejiang university college of medicine from Jan.2000 to Jan.2009.All patients were confirmed by both surgery and pathology.Results:There were 104 patients,64 men and 40 women,with age ranged from 13 to 77.The mean age was 42.The course of disease was between one week and ten years,with an average of 17.3 months.Ten of the patients had multiple tumors, the others had only one lesion.Five patients had a family history,and ten patients had final diagnosis of VHL disease.The clinical presentations mainly depend on the position and volume of the lesion.86 patients were diagnosed exactly before operations,with accurracy rate of 83%.Pre-operative embolization of feeding arteries was performed in2cases.Total tumor removal was accomplished in 91 cases,partial removal in 13 cases and adjuvant gamma knife radiosurgery in 4 cases.69 patients were followed up,with a mean period of 36 months.The KPS scores showed above 80 in 68 patients while the other 11 had less.Conclusion:CT and MRI are necessary for diagnosis.DSA is valuable for the suspected cases.Pre-operative endovascular embolisation,controlled lowering of blood pressure during operation and postoperative sterotherapy play an important role in treatment of solid tumors.The treatment of parenchymal,familial HB,and multiple HB especially in the medial sites such as the brain stem and spinal cord remains an difficulty in neurosurgery.Familial HB involves multiorgan onsets and easy to relapse,which should be paid more attention by the clinical physicians.