血管母细胞瘤35例临床分析

目的 通过对颅内血管母细胞瘤(HBs)的影像学、病理和治疗分析,探讨HBs的诊断、形成原因和疗效. 方法 皖南医学院弋矶山医院神经外科自2005年1月至2010年1月应用肿瘤切除术治疗35例颅内HBs患者,根据影像学表现分为大囊小结节型、小囊大结节型和完全实质型.分析患者的临床资料,对肿瘤标本行HE染色和免疫组化染色检测NSE和CD34的表达. 结果 本组肿瘤全切除34例,次全切除1例,术后无死亡.出院后28例获得随访,随访时间3个月至3年.其中1例复发(大囊小结节型)行伽玛刀治疗.HE染色显示纤细血管相互吻合成网状,分隔脂质胞浆；免疫组化染色显示不同类型肿瘤CD34阳性细胞数比较差异无统计学意义(P＞0.05).大囊小结节型肿瘤NSE阳性细胞数最多,小囊大结节型其次,实质型最少,差异均有统计学意义(P＜0.05). 结论 HBs无特殊临床表现,影像学表现是主要诊断方法.治疗首选手术切除,手术全切除是降低复发的关键.HBs囊腔的形成与肿瘤间质细胞密切相关.

Diagnosis and surgical treatment of 35 patients with hemangioblastomas

Objective To analyze the imaging and pathological characteristics, as well as treatment strategies of intracranial hemangioblastomas (HBs),and explore the advancement of diagnosis,etiopathogenisis and treatment of HBs. Methods Thirty-five patients with intracranial HBs,admitted to our hospital and performed tumor resection from January 2005 to January 2010,were chosen in our study; all patients were divided into type of big cystic HBs with a small mural nodule (n=19),type of small cystic HBs with a big nodule (n=9) and type of solid HBs (n=7) by imaging features.The clinical manifestations,imaging findings and surgical methods were retrospectively analyzed; the expressions of NSE and CD34 in these tumor samples were detected by HE staining and immunohistochemical staining.Results All patients were treated by surgery; total resection was achieved in 34 and subtotal resection in 1; no death occurred after the surgery.Twenty-eight patients were followed up for 3 months to 3 years after discharge; recurrence appeared in 1 patient with big cystic HBs with a small mural nodule and Gamma knife treatment was performed.No significant difference was observed in the numbers ofCD34+cells between each 2 types of patients (P＞0.05).The numbers of NSE positive cells between each 2 types were statistically significant (P＜0.05). Conclusion There were no specific clinical manifestations of HBs.Diagnosis was mainly according to imaging features.Treatment of HBs with total resection is just the first selection and the key to reduce palindromia; the formation of HBs cysts is closely related to tumor stromal cells.