颅内血管母细胞瘤的临床研究

目的 探讨颅内血管母细胞瘤（HB）的临床特点及次全切除术后的综合疗效。方法 采用回顾性分析方法，对经手术完全切除的38例（手术组）及手术切除有残灶、术后辅以放疗的25例（综合治疗组）颅内血管母细胞瘤进行临床分析和疗效总结。主要临床症状为颅内压增高（35例）、小脑机能障碍（20例）及颅神经损害（8例）。52例病灶位于小脑，肿瘤病理组织学特点由毛细血管网和间质细胞构成。结果 综合治疗组5例复发，4例死于复发，22例非脑干区术后残留肿瘤照射剂量50～55Gy；其5、10和15年生存率分别为100％、92％和89％。手术组6例复发，3例死于复发；其5、10、15年生存率分别为97％、85％、79％。结论 颅内血管母细胞瘤好发于成人小脑部位，术后有残留者辅以放疗可能会减少复发。

Clinical study of intracranial hemangioblastoma

Objective To investigate the clinicopathological characteristics of intracranial hemangioblastoma(HB) and its effect with postoperative(subtotal tumor resection) radiotherapy.Methods Sixty-three such patients 38 were analyzed retrospectively.Thirty-eight patients received total tumor resection alone(surgery alone group),the other 25 received subtotal tumor resection plus postoperative radiotherapy(combined treatment group).The most common clinical symptom was intracranial hypertension(35 patients),followed by cerebellar ataxia(20 patients) and cranial nerve injury(8 patients).Eight percent of tumor was located at the cerebellum.Stromal cells woven capillary vascular network featured the tumor under microscopy.Results Five of the 25 patients in the combined group suffered tumor relapse,with a rate of 20%,of whom 4 patients died of recurrence.Six in the 38 patients treated by surgery alone developed relapse,with a rate of 16%,of whom 3 patients died of recurrence.The 5-,10-and 15-year survival rate was 100%,92%,89% in the combined group and 97%,85%,79% in the surgery alone group,respectively.Conclusion Intracranial hemangioblastoma,a benign tumor,mainly occurs in adult,almost always located in the cerebellum.Tumor relapse is the main cause of death.Postoperative radiotherapy can lower the incidence of tumor relapse who have received subtotal tumor resection.