Clinical Data Analysis And Long Term Following Up Results In 312 Cases With Central Nervous System Hemangioblastomas: Experience In One Institution

OBJECTIVE: The impact of neurosurgical progresses on patients with central nervous system hemangioblastomas (HBs) in von Hippel-Lindau (VIII.) syndrome or sporadic disease was analyzed. METHODS: The surgical pathology database of our institution was searched to identify 312 patients with hi stological1y verified CNS hemangioblastomas from May 1974 through Dec 2003. According to three periods, a comparison was conducted focusing on the diagnosis and treatment. RKSULTS: Our studysample comprised 188 men and 124 women, with mean age 39 years old. 19 cases (6. 09%) hatl family history and 20 cases ( 6. 4 1%) were associated with VHL. disease. The mean duration of symptoms were 16.2 months. These 312 patients presented with 331 hemangioblastomas (20 patients had multiple lesions). The rate of correct preoperative diagnosis was 42% , 58% and 92% in fist period (May 1974 Jul 1989) , second period (Auk 1989 Dec 1996) and third period (Jan 1997~Dec 2003) respectively. The rate of gross total remova1 was 83% , 80. 5% and 97.9% in fist period , second period and third period respectively .Comparing the preoperative and postoperativeneurological function in discharge, 89% , 84.4% and 78.4% improved in three periods respectively. 8%, 7. 8%, 3. 1% died after operations in three periods respectively. The rate of KPS>80' in three periods was 88.5%, 92.1%, 79.7% when following up from 4 months to 13 years. CONCLUSIONS: MR I is the most important method for the diagnosis of HBs. DSA is valuable for the suspected cases and preoperativeEmbolization. Surgical outcomes for patients with CNS hemangioblastomas are favorable. However, management of hemangioblastomas is a more difficult and prolonged endeavor for patients with VHL. syndrome or sol id tumors. Neurosurgeons should pay more attentions to VHL disease.