31例儿童髓母细胞瘤临床特征和预后危险因素分析

摘要

Objective To analyze and discuss the clinical features and prognostic risk factors of medulloblastoma in children.Methods Thirty-one patients with pathological diagnosis of medulloblastoma who were treated in our hospital from August 2009 to November 2016 were divided into the death group and survival group,relapsed group and routine group,and the effects of age,gender,tumor size,surgical resection,immunohistochemistry,M stage,radiotherapy and chemotherapy,and other factors on prognosis were analyzed retrospectively.Results As of April 2018,the follow-up time was 1.4 to 8.7 years and the median follow-up time was 2.9 years.Among them,9 died,16 survived,and 6 lost.The age of onset was between 1 year 7 months and 11 years 9 months.The first symptoms were mostly headache (67.7％),vomiting (83.9％),unstable walking (32.3％),and partial forcing head (12.9％) and diplopia (6.5％).28 cases of the tumors were located in the cerebellar vermis,1 case left cerebellar hemisphere,1 case left cerebellopontine angle,1 case right cerebellar hemisphere.Imaging examination revealed the average diameter was 41 mm.All of the children included in the study underwent surgical resection,of which 18 cases (58.1％) were totally resected and 13 cases (41.9％)were mostly resected.3 cases only had radiotherapy and 3 cases only had chemotherapy after surgery.5 patients relapsed,of which 3 gave up treatment,1 had secondary surgery,radiotherapy and chemotherapy,1 received radiotherapy and chemotherapy again.During follow-up,early cerebrospinal fluid dissemination in 2 cases,cerebrospinal fluid dissemination after recurrence in 1 case,early metastases occurred in 6 cases and bone metastases occurred after recurrence in 1 case.The chi-square test indicated that there were statistical differences between the survival and death group in terms of the surgical-radiotherapy interval,radiotherapy and M stage (P＜ 0.05).Univariate analysis revealed that the surgical-radiotherapy interval,radiotherapy,and M stage were prognostic risk factors(P=0.007,0.040,0.002),and those who did not receive radiotherapy and chemotherapy after surgery were more likely to have recurrence (P =0.006).The most common adverse reactions after surgery were facial paralysis and weakening of muscle strength.Only 1 patient suffered mental impairment after radiotherapy.The most common side effects of chemotherapy were vomiting,hematologic toxicity,and infection.All infections were cured after active treatment.Conclusions Childhood medulloblastoma has a high degree of malignancy,rapid progress,and is easy to transfer.It needs to be early diagnosed and clearly staged.Surgical resection is the basic treatment.Children can tolerate radiotherapy and chemotherapy,and radiotherapy should be performed after surgery as soon as possible.Reasonable radiotherapy and chemotherapy can reduce recurrence,which are important to improve the prognosis.%目的 分析并探讨儿童髓母细胞瘤的临床特征及预后相关危险因素.方法 选取2009年8月-2016年11月南京市儿童医院收治的经病理诊断为髓母细胞瘤的31例患儿,分为死亡组和存活组、复发组和常规组,回顾性分析了年龄、性别、肿瘤大小、切除程度、免疫组化、M分期及放化疗等因素对其预后的影响.结果 截至2018年4月,随访时间1.4～8.7年,中位随访时间2.9年,其中死亡9例,存活16例,失访6例.发病年龄在1岁7个月～11岁9个月,首发症状多为头痛(67.7％)、呕吐(83.9％)、行走不稳(32.3％),部分可出现强迫头位(12.9％)及复视(6.5％).全部研究病例中,28例患儿肿瘤位于小脑蚓部,1例位于左侧小脑半球,1例左侧桥小脑角区,1例右侧小脑半球,影像学检查提示肿瘤平均直径41 mm.所有患儿均行肿瘤切除手术,其中肿瘤全切除18例(58.1％),大部分切除13例(41.9％).3例术后仅化疗未放疗,3例术后仅放疗未化疗.5例患儿复发,其中3例放弃治疗,1例再次手术及术后重新放化疗,1例仅重新予放疗及化疗.随访期间,2例发病早期出现脑脊液播散,1例复发后出现脑脊液播散,6例早期出现肿瘤转移,1例复发后出现骨转移.卡方检验提示存活组和死亡组患儿在手术放疗时间间隔、是否放疗及M分期三个方面具有统计学差异(P＜0.05).单因素分析发现手术放疗时间间隔、放疗及M分期是影响预后的危险因素(P值分别为0.007、0.040、0.002),术后未行放疗及化疗者更易复发(P=0.006).术后最常见的不良反应是面神经瘫痪和肌力减弱,仅1例在放疗后出现智力受损,化疗最常见的不良反应为呕吐、血液系统毒性及感染.结论 儿童髓母细胞瘤恶性程度高,进展迅速,早期易转移,需尽早明确诊断并分期.手术切除是髓母细胞瘤的基本治疗手段,患儿对放化疗耐受性可,术后无禁忌证,应尽早放疗.合理联合化疗可降低复发率,对改善预后有重要意义.