投稿-结节性硬化症合并室管膜下巨细胞星形细胞瘤手术治疗的临床预后和生活质量评估.docxVIP

结节性硬化症合并室管膜下巨细胞星形细胞瘤手术治疗的远期预后和生活质量评估刘玉含王军梅 姜涛李春德 田永吉【摘要】目的结节性硬化症合并室管膜下巨细胞星形细胞瘤患者手术治疗的远期临床预后及生活质量评估。 方法 回顾性分析2005年12月至2012年2月首都医科大学附属北京天坛医院神经外科收治的19例?患者的临床资料，及随访的预后结果，随访时间为5-11年，平均6.9年。1例有明确家族史，4例患儿术前视力下降，2例患儿有智力障碍，5例患儿合并有癫痫。结果19例患儿均接受手术治疗切除颅内肿瘤，1例失访，17例全切未再复发；2例肿瘤部分切除均接受二次手术治疗。2例合并对侧肿瘤未切除,1例术后对侧肿瘤有增大，服用雷帕霉素后肿瘤缩小，另1例为失访患者；1例术前行脑室腹腔分流手术，2例术后行分流手术，其中1例为双侧脑室腹腔分流手术。1例患者术后5年死于突发颅内出血，1例患者术后颅内出血二次手术后出现癫痫及视力下降症状。1例患儿术后出现肾脏肿瘤，于外院手术治疗。4例患者术后在家休养，13例患儿目前能生活自理、上学或者工作。结论室管膜下巨细胞星形细胞瘤是良性肿瘤，肿瘤全切率较高,部分患者会有肿瘤残留或者对侧脑室肿瘤未能切除，患者可能出现记忆力下降、癫痫、肿瘤不能全切除等情况。【关键词】 结节性硬化症 室管膜下巨细胞星形细胞瘤 手术 预后 生活质量long-term prognosis and life quality of subependymal giant cell astrocytoma patients associated with tuberous sclerosis complexLIU Yu-han, WANG Jun-mei, JIANG Tao, LI Chun-de, TIAN Yong-ji[Abstract] ObjectiveTo investigate long-termprognosis and life quality in subependymal giant cell astrocytoma patients associated with tuberous sclerosis complex who received tumor removal in our hospital. MethodsFromDecember 2005 to February 2012, 19 patients received tumor removal in Beijing Tiantan Hospital. One patient had family history of tuberous sclerosis, four patients had impaired vision preoperatively, two patients were diagnosed as mental retardation, and five patients had epilepsy seizure. Results One patient was lost of follow-up who had double intracerebral tumors and tumor residue in contralateral ventricle. Another patient with contralateral tumor residue received oral rapamycin treatment. Two tumors were partial removed and received secondary operations. There was no tumor relapse in patients with total removal. Two postoperativeinternal shunts were performed and one patient received ventriculoperitoneal shunts in double sides. One patients died of suspected intracranial bleeding. One patient received secondary surgery because of intracranial hematoma after transcortical approach. This patient had coma for about one month and complained of new onset epilepsy. One patient had kidney tumor which