儿童髓母细胞瘤临床病理分析

Clinical pathological analysis of children with medulloblastoma

目的探讨儿童髓母细胞瘤(MB)的病理特征及其治疗与预后,分析其预后与病理类型的相关性。方法回顾性分析60例MB患儿的病理、治疗结局、及其他临床资料。结果 60例MB患儿均为高级别肿瘤,病理类型主要为经典型45例(75.00%),其他各亚型15例(25.00%)。显微手术全切除48例(80.00%),次全切除9例(15.00%),大部切除3例(5.00%)。术后放疗27例,化疗6例,放疗联合化疗5例。38例患儿获随访,2年存活率与无病生存率为63.16%和55.26%,5年存活率与无病生存率为26.32%和18.42%。大细胞型和间变型MB生存时间较短,促结缔组织增生/结节型生存时间较长;手术切除范围为全切除的患儿生存时间较长,部分切除患儿生存时间较短;放疗或化疗患儿的生存时间长于没有放、化疗的患儿,差异均有统计学意义(P均<0.05)。结论儿童MB多为高级别肿瘤,预后差,病理组织学分型与预后密切相关。显微外科全切除是治疗首选。术后放、化疗可有效延长其生存期。

Objectives To explore the pathological characteristics, treatment and prognosis of medulloblastoma （MB） in children. Methods Pathological characteristics, treatment outcomes and other clinical data were retrospectively analyzed in 60 children with MB. Results The MB in all 60 children were high-grade tumor. The pathological type was mainly classic （45 cases, 75.00%） and other subtypes were seen in 15 cases （25.00%）. Forty-eight （80.00%） children had total resection by microsurgery, 9 children （15.00%） subtotal resection, and 3 children （5.00%） partial resection. Twenty-seven children were treated by radiotherapy after surgery, 6 children were treated by chemotherapy after surgery, and 5 children were treated with radiotherapy combined chemotherapy after surgery. Thirty-eight cases were followed up. The 2-year survival and disease-free survival rates were 63.16% and 55.26%. The 5-year survival and disease-free survival rates were 26.32% and 18.42%. The children with des- moplastic/nodular type survived significantly longer than the children with large cell anaplastic. The children with total resection survived significantly longer than the children with partial resection. The children with radiotherapy or chemotherapy survived significantly longer than the children without chemotherapy （P〈0.05）. Conclusions MB in most of children is high-grade tumors and the prognosis is poor. The pathological type is related with prognosis. Microsurgical total resection is the first choice of treatment. Postoperative radiotherapy and chemotherapy can increase the survival rate.