结节性硬化症并室管膜下巨细胞星形细胞瘤手术患者远期预后和生活质量评估  

Long-term Prognosis and Life Quality of Subependymal Giant Cell Astrocytoma Patients with Tuberous Sclerosis Complex

作　　者：刘玉含[1] 王军梅[1] 杜江[1] 李春德[1] 姜涛[1] LIU Yu-han WANG Jun-mei DU Jiang LI Chun-de JIANG Tao(Beijing Neurosurgical Institute Beijing Tiantan Hospi- tal, Capital Medical University, Beijing 100051, China)

机构地区：[1]首都医科大学附属北京天坛医院北京市神经外科研究所,北京100051

出　　处：《临床误诊误治》2016年第10期12-15,1,共4页Clinical Misdiagnosis & Mistherapy

基　　金：国家科技支撑计划项目(2013BAI09B03)

摘　　要：目的 总结结节性硬化症（tuberous sclerosis complex,TSC）并室管膜下巨细胞星形细胞瘤（subependymal giant cell astrocytoma,SEGA）手术患者远期预后及生活质量。方法 回顾性分析2005年12月—2012年2月我院神经外科手术治疗的19例TSC并SEGA临床资料。结果 19例均行手术治疗,采用经额皮质造瘘入路15例,经胼胝体侧脑室入路4例,术前行脑室腹腔分流术及脑室穿刺外引流各1例。17例行肿瘤全切未再复发,2例行肿瘤部分切除后因残余肿瘤增大,而接受二次手术治疗。本组术后随访5~11年,平均6.9年,其中17例长期存活,死亡及失访各1例。1例术后5年死于突发“脑干出血”,1例术后颅内出血二次手术后出现癫痫及视力下降;1例术后出现肾脏肿瘤于外院手术切除。2例双侧脑室肿瘤均予一侧切除,1例术后对侧肿瘤增大,给予西罗莫司治疗后肿瘤缩小;另1例失访。长期存活患者中,4例术后在家休养,13例目前能生活自理。结论 提高对TSC并SEGA的认识,密切观察患者颅内病变情况,以早期发现SEGA并手术,以取得较好预后;辅助应用m TOR抑制剂可一定程度抑制SEGA生长。Objective To investigate long-term prognosis and life quality of subependymal giant cell astrocytoma （SEGA） patients with tuberous sclerosis complex （TSC）. Methods Clinical data of 19 SEGA patients with TSC during December 2005 and February 2012 was retrospectively analyzed. Results The 19 patients received surgical therapy, and 15 patients were removed through transcortical approach and 4 patients were through transcallosal approach. Presurgical ventriculoperitoneal shunt and external ventricular drainage were performed for 2 patients respectively. No recurrence was found in 17 patients after total resection, and 2 patients received secondary operations because of enlarged residual neoplasm. All patients were followed up for 5-11 years, and average follow-up time was 6.9 years in whom 17 patients were long-term surviving, and one died and another lost follow-up. One patient died of brain stem hemorrhage in the postoperative 5th year; one patient had onset epilepsy and impaired vision after receiving secondary surgery because of postoperative intracranial hematoma; one patient had kidney tumor, and was removed to another hospital. The 2 patients with bilateral cerebral ventricle neoplasms were treated with unilateral resection, and then one patient had contralateral tumor enlargement, and tumor was decreased after Sirolimus treatment; another patient lost follow-up. Among 17 patients with long-term surviving, 4 patients were at home, and 13 patients could self-care. Conclusion Clinicians should improve understanding of SEGA patients with TSC and closely observe intracalvarinm lesions of TSC patients so as to perform surgery as early as possible for good prognosis, roTOR inhibitors of assisted application can inhibit tumor growth in a certain degree.

关 键 词：结节性硬化症 室管膜下巨细胞星形细胞瘤 外科手术 预后 生活质量 

分 类 号：R739.4[医药卫生—肿瘤] R596.1[医药卫生—临床医学]