小脑性缄默与儿童髓母细胞瘤

Cerebellar mutism and childhood medulloblastoma

目的：探讨小脑性缄默（CM）与髓母细胞瘤（MB）复发及原发肿瘤位置的相关性。方法回顾性分析2011年11月至2015年4月收治的114例3岁以上MB患儿的临床资料。结果114例患儿中，男77例、女37例，初次发病中位年龄84.7月（36.4～184.7月），其中48例为复发病例。22例发生CM，发生率为19.3%（22/114）；其中非复发及复发MB患儿CM发生率分别为19.7%（13/66）及18.8%（9/48），二者间差异无统计学意义（P=0.899）；原发肿瘤位于第四脑室者的CM发生率17.6%（9/51），小脑蚓部为7.1%（1/14），第四脑室及小脑蚓部同时受累者21.4%（3/14），第四脑室合并其他部位受累者45.5%（5/11），小脑蚓部合并其他部位受累者50.0%（4/8），非第四脑室及小脑蚓部受累者无CM发生。CM发生率在原发肿瘤位置累及第四脑室和/或小脑蚓部与未累及第四脑室和/或小脑蚓部患儿间的差异有统计学意义（P=0.039）。结论 CM发生与MB患儿复发无相关性。原发肿瘤侵犯第四脑室和/或小脑蚓部者易发生CM。

Objective To investigate the relationships among cerebellar mutism （CM）, relapsed medulloblastoma （MB） and the primary tumor location.MethodsA retrospective analysis was conducted in 114 children over 3 years old with MB from November 2011 to April 2015.ResultsThe median onset age was 84.7 months （36.4 to 184.7 months） in 114 children with MB （77 boys and 37 girls）, of whom there were 48 cases of recurrence. There were twenty two cases of CM and the overall incidence of CM was 19.3% （22/114）. The incidence of CM was 19.7% （13/66） in non-recurrent cases and 18.8% （9/48） in recur-rent cases, and there was no signiifcant difference between two groups （P=0.899）. The incidence of CM was 17.6% （9/51） in cas-es with primary tumor in the fourth ventricle, 7.1% （1/14） in cases with primary tumor in the cerebellar vermis, 21.4% （3/14） in cases with primary tumor in both fourth ventricle and cerebellar vermis, 45.5% （5/11） in cases with primary tumor in fourth ven-tricle and other parts of the brain, and 50.0% （4/8） in cases with primary tumor in cerebellar vermis and other parts of the brain. No CM incidence was observed in cases with primary tumor in central nerve system except for the fourth ventricle and cerebellar vermis. The incidence of CM between the cases with fourth ventricle/cerebellar vermis involvement and those without fourth ventricle/ cerebellar vermis involvement had signiifcant difference （P=0.039）. ConclusionsThere is no relationship between CM and relapsed MB. Children with MB whose primary tumor is located in the fourth ventricle and/or the cerebellar vermis is susceptible to CM.