中枢神经系统血管母细胞瘤312例临床分析及长期随访

Clinical data analysis and long time following up in 312 cases of central nervous system hemangioblatomas

目的探讨医学发展对本病诊治的影响.方法回顾性总结分析上海华山神经外科集团医院1974年5月至2003年12月收治的312例中枢神经系统血管母细胞瘤病人的临床资料,分3个时期比较其诊断与治疗进展情况,说明近年来医学发展对本病诊治的影响.结果本组312例中,男188例,女124例,男:女=1.52:1;年龄14～72岁,平均39岁;多发性肿瘤20例,共331个肿瘤,最常见于小脑半球或蚓部,其次是脊髓,亦可偶见于幕上;有明确家族史者19例(6.1%),确诊VHL病20例(6.4%).在前期(1974年5月至1989年7月)CT和气脑、脑室造影是主要诊断方法,术前确诊率42%;中期(1989年8月至1996年12月)和后期(1997年1月至2003年12月)CT和MRI是主要诊断手段,术前确诊率分别为58%、92%;后期还开展术前栓塞治疗.肿瘤全切率三个时期分别为83%、80.5%、97.9%,术后死亡率分别为8%、7.8%、3.1%.长期随访KPS＞80分者三组分别为88.5%、92.1%、79.7%,死亡率分别为8.2%、0、3.1%.结论本病诊断主要依靠MRI,对可疑病人应做DSA检查,但早期诊断仍存在问题;手术治疗可靠有效,肿瘤全部切除可治愈本病;但实质性、家族性、多发性中枢神经系统血管母细胞瘤(HB)特别是位于脑干、脊髓时治疗仍较困难;家族性HB(VHL病),因累及脏器多,易复发,愈后较散发性差,而临床医生应加强对VHL病的重视.

Objective The impact of neurosurgical progresses on patients with central nervous system hemangioblastomas (HBs) in von Hippel-Lindau (VHL) syndrome or sporadic disease was analyzed. Methods The surgical pathology database of our institution was searched to identify 312 patients with histologically verified CNS hemangioblastomas from May 1974 to Dec 2003.According to three periods, a comparison was conducted focusing on the diagnosis and treatment. Results Our study sample comprised 188 male and 124 female, with mean age of 39 years old. 19 cases(6.1%) had family history and 20 cases(6.4%) 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(Aug 1989~Dec 1996) and third period(Jan 1997~Dec 2003) respectively. The rate of gross total removal was 83%, 80.5% and 97.9% in first period, second period and third period respectively .Comparing the preoperative and postoperative nervous system function at discharge, 89%, 84.4% and 78.4% improved in the three periods respectively. 8%, 7.8%, 3.1% died after operations in the 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 MRI is the most important method to the diagnosis of HBs. DSA is valuable for the suspected cases and preoperative embolization.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 solid tumors. Neurosurgeons should pay more attentions to VHL disease.