成人髓母细胞瘤11例临床分析

Clinical Analysis of Eleven Cases of Medulloblastoma in Adult

背景与目的:髓母细胞瘤是好发于儿童的恶性的胚胎性神经上皮肿瘤,成人则较为少见。本文总结报道成人髓母细胞瘤的诊治方法及治疗效果。方法:回顾性分析本院2003年1月至2010年1月的11例成人髓母细胞瘤的临床表现、影像学特点、治疗措施并进行随访。结果:11例表现为颅内压增高和小脑功能受损症状。均行MR检查,位于小脑蚓部4例,小脑半球4例,小脑蚓部+小脑半球2例,桥小脑角1例。表现为T1低或稍低信号,T2和水抑制多为高或稍高信号,增强肿瘤不均匀中度强化。行手术全切除6例,次全切除5例。术后病灶局部加全中枢神经系统放疗+化疗6例,行头颅病灶局部放射治疗1例。7例患者获术后定期MR检查、随访1～8年,3例失访,1例术后放弃治疗出院。术后MR复查证实中枢神经系统原位复发并脊髓转移2例。术后2年生存率100%,5年生存率50%。结论:成人髓母细胞瘤发病率低,临床表现及放射学特点与儿童髓母细胞瘤有许多不同点,诊断相对困难,综合治疗是主要治疗手段,治疗效果不理想。

BACKGROUND OBJECTIVE： Medulloblastoma is a malignant embryonal neuroepithelial tumor that occurs commonly in children but rarely in adults.In this article,we summarized and reported the methods of diagnosis,management and the therapeutic efficacies of medulloblastomas in adults.METHODS： Clinical manifestations,imaging features,operating measures of Eleven patients with medulloblastoma treated in our hospital from January 2003 to January 2010 were retrospectively analyzed.RESULTS： Among 11 cases,increased cranial pressure and cerebellar symptoms were the most common manifestations.Magnetic resonance scanning was performed in all cases.Among 11 cases,4 tumors were located in vermis cerebellum,4 in cerebellar hemisphere,2 in both vermis cerebellum and cerebellar hemisphere and 1 were in cerebellopontine angle,respectively.The tumors were hypo-intense on T1-weighted MR images and hyper-intense on T2-weighted MR images.The tumors were moderately contrast-enhanced.Total resection was achieved in 6 cases and subtotal resection in 5.Six patients received craniospinal radiotherapy and chemotherapy.One case only received encephalic radiotherapy.Seven cases were followed up for 1 to 8 years.The 2-year survival is 100% while the 5-year survival rate is 50%.CONCLUSIONS： The incidence of medulloblastoma in adult is low.The clinical presentations and the imaging features are different from those in child.It is difficult to diagnose.The combined therapy is the main methods and the therapeutic effect of them remains suboptimal.