成人髓母细胞瘤的临床治疗及预后分析

Analysis of clinical treatment and prognosis of adult medulloblastoma

目的探讨成人髓母细胞瘤（MB）的治疗方式及预后。方法筛选经手术及病理证实为MB的成人患者163例，回顾性分析其临床表现、影像学、治疗方式及预后特点。其中包括男性108例，女性55例，平均28．6岁。结果患者临床表现主要有头痛、恶心及呕吐、头晕、步态不稳、视力减退、复视、听力下降、小脑危象等。术后肿瘤全切90例，近全切67例，部分切除6例。共随访患者160例，其中生存121例，死亡39例，生存时间为7～170个月，中位生存期（127．4-6）个月（95％CI115．3—138．7），5年生存率73．1％（120／163）。接受全脑一脊髓放疗患者组与全脑或局部放疗患者组生存期比较，差异具有统计学意义（中位生存时间分别为44个月和34个月，）（χ2=8．712，P=0．003）。结论手术切除结合早期足量的全脑一脊髓放疗可有效治疗成人MB，肿瘤复发和转移是造成患者预后差的主要因素。

Objective To analyze the clinical manifestations, treatment and prognosis of adult medulloblastoma （MB）. Methods A total of 163 cases of adult MB, confirmed by surgical and pathological diagnosis, were retrospectively analyzed about the clinical manifestations, imaging, treatment and prognosis. There are 108 males and 55 females in the group, whose average age was 28.6-year-old. Results The main clinical manifestations include headache, nausea, vomiting, dizziness, gait disturbance, hypopsia, diplopia, hearing loss and cerebellum crisis. Gross total resection was achieved in 90 cases, subtotal resection in 67 cases and biopsy in 6 cases. Survival time from surgery to progression or death or the last date of follow-up were measured and estimated by the Kaplan-Meier method. Among a total of 160 follow-up patients with MB, postoperative overall survival time was from 7 to 170 months, median survival time was （127 ＋6） months （95% CI 115.3--138.68）. The 5-year overall survival time of adult MB patients was 73.1% （ 120/163 ）. The log-rank test was used to compare the significance of the following prognostic variables. Among all clinical factors, patients undergoing the craniospinal irradiation had a significantly better survival rate than those without this treatment （44 months vs 34 months, χ2 = 8. 712, P = 0. 003 ）. Condusion Good therapeutic effect of MB be achieved by adopting surgical resection with early enough craniospinal irradiation. Recurrence and metastasis are the two main factors for bad prognosis in adult MB.