原发性脊髓间变性室管膜瘤的临床特点和诊治

摘要

Objective To discuss the clinical features,treatment and outcomes of primary spinal anaplastic ependymomas (PSAE).Methods Between January 2008 and October 2015,a series of patients with PSAE were surgically managed at Department of Neurosurgery,Beijing Tiantan Hospital,Capital Medical University and enrolled into this retrospective study.This series included 11 consecutive patients (6 females and 5 males) accounting for 1.4％ (11/765) of all case of spinal ependymomas during the same period.The average age at presentation was 22.7 ± 12.4 years (range:8-44 years).The mean preoperative course was 3 months (range:1.5-18.0 months).All those lesions were surgically treated and pathologically verified.Results Gross total resection was achieved in 10 patients,and subtotal resection in 1 patient.Six patients received adjuvant radiation therapy and I patient received chemotherapy.During the follow-up lasting an average of 48.9 ± 7.9 months (range:17-110 months),excellent outcome was achieved in 4 cases,3 remained stable and 4 deteriorated including 2 died.Five patients developed recurrence after 25.0 ± 7.2 months (range:4-39 months),and 3 patients underwent a second operation.Conclusion PSAE seems to be a type of rare high-grade ependymomas and characterized by short preoperative course,fast progress,high rate of recurrence and poor outcome.%目的 探讨原发性脊髓间变性室管膜瘤(PSAE)的临床特点、治疗及预后.方法 回顾性分析2008年1月至2015年10月于首都医科大学附属北京天坛医院神经外科行显微外科手术治疗的PSAE患者的临床资料,共11例,占同期脊髓室管膜瘤患者总数的1.4％ (11/765).11例中,男/女=5/6,发病年龄为8～44岁,平均(22.7±12.4)岁.发病至手术时间为1.5 ～18.0个月,中位时间为3.0个月.术后6例患者接受放疗,1例接受化疗.结果 11例患者中,肿瘤全切除5例,近全切除5例,大部分切除1例,无手术相关并发症.术后随访17～110个月,平均(48.9±7.9)个月.5例复发,复发距离手术时间为4 ～ 39个月,平均(25.0±7.2)个月,其中3例再次接受手术治疗,2例由于一般状况较差未手术.至随访期末,4例临床症状改善,3例无明显变化,4例症状加重(其中2例死亡).结论 PSAE是一种罕见的高级别室管膜瘤,病程较短,进展较快;术后易复发,预后差.