儿童髓母细胞瘤神经轴外转移的临床特点分析

Clinical feature analysis of medulloblastoma with extraneural metastasis in children

目的总结儿童髓母细胞瘤神经轴外转移的病例特点。方法回顾性分析首都医科大学附属北京世纪坛医院2010年4月至2019年4月收治的616例髓母细胞瘤患儿的临床资料,其中11例出现了神经轴外转移。描述性分析患儿的起病年龄、神经轴外转移的部位及时间、病理及分子分型、治疗及预后情况。采用t检验分析神经轴外转移患者和非转移患者的血生化指标之间的差异。结果截至2020年2月,中位随访时间16个月(3~69个月)。随访的616例患儿中有11例出现了神经轴外转移,发病率约为1.8%,其中男8例,女3例。确诊时的中位年龄为6岁(2~10岁),出现神经轴外转移时的中位年龄为7岁(2~12岁)。神经轴外转移多发生在术后0.5~38.0个月,转移部位依次为骨(6例)、骨髓(3例)、肺(3例)、盆腔(2例)和腹腔(1例)。另外,神经轴外转移患儿的血清乳酸脱氢酶(LDH)水平为(2298.00±1570.70)U/L,神经元特异性烯醇化酶(NSE)水平为(201.00±68.34)μg/L,均高于未发生轴外转移的患儿[(249.50±46.28)U/L和(22.80±7.12)μg/L,均P<0.05]。神经轴外转移患儿部分接受化疗,但大多选择终末期姑息治疗,生存期多低于10个月。结论尽管髓母细胞瘤患儿发生神经轴外转移的发生率很低,但其预后极差,多在1年内死亡;最常见的转移部位为骨,其次为骨髓和肺,可能与脑脊液播散及LDH、NSE水平增高有关。

Objective Summarizing the clinical characteristics of extraneural metastasis in childhood medulloblastoma.Methods A total of 616 cases with medulloblastoma treated in Beijing Shijitan Hospital from April 2010 to April 2019 were analyzed retrospectively,among which 11 cases developed extraneural metastasis.The age of onset,location and time of extraneural metastasis,pathological and molecular typing,treatment and prognosis were descriptively analyzed.The differences of blood biochemical indexes between medulloblastoma cases with and without extraneural metastasis were statistically analyzed by t test.Results As of February 2020,the median follow-up period was 16 months(ranging from 3 to 69 months).Eleven cases,including 8 males and 3 females,were diagnosed with extraneural metastasis,with the incidence being about 1.8%.The median age of medulloblastoma was 6 years(2-10 years),and the median age at presentation of extraneural metastasis was 7 years(2-12 years).Extraneural metastasis occurred from 0.5 months to 38.0 months after the operation,and the affected location includes bone(6 cases),bone marrow(3 cases),lung(3 cases),pelvis(2 cases)and abdominal cavity(1 case).In these patients,the range of lactic dehydrogenase(LDH)was(2298.00±1570.70)U/L and neuron-specific enolase(NSE)was(201.00±68.34)μg/L,which were significantly higher than those in patients without extraneural metastasis[(249.50±46.28)U/L and(22.80±7.12)μg/L,all P<0.05].Partial patients were treated with chemotherapy,while the majority of them were treated with palliative treatment in the terminal stage,with the survival period mostly less than 10 months.Conclusions Although there is a low incidence of extraneural metastasis in medulloblastoma pediatric patients,the prognosis of these patients with extraneural metastasis is poor and most of them would die within one year.The most common sites include bone,followed by bone marrow and lungs,which may be related to the spread of cerebrospinal fluid and the increased levels of LDH and NSE.