Department of Pediatrics, Cancer Research Institute, New York, USA.
Department of Pediatrics, Hallym University College of medicine, Chuncheon Sacred Heart Hospital, Chuncheon, Korea.
World J Pediatr. 2017 Aug;13(4):367-373. doi: 10.1007/s12519-017-0044-3. Epub 2017 May 27.
Although craniospinal irradiation (CSI) of 2340 cGy plus tumor booster with chemotherapy have been established as a standard treatment of childhood average-risk (AvR) medulloblastoma (MBL) in Western counties, there are a few recent reports in outcomes of AvR MBL using this strategy in Korean and other Asian children. We investigated the outcome of the Korean children with AvR MBL who were treated with CSI <2500 cGy and chemotherapy.
Between January 2001 and December 2010, clinical characteristics and outcomes of 42 patients who were diagnosed with AvR MBL postoperatively and treated with radiation including CSI <2500 cGy and chemotherapy in Seoul National University Children's Hospital were analyzed.
Their median age was 9 years (range: 3-18.8), and 29 were male. Histological subtypes were classic type in 28 patients, nodular/desmoplastic in 7, and large cell/anaplastic (LCA) in 7. All the patients received adjuvant radiotherapy (CSI with median 2340 cGy and booster) and multiagent chemotherapy as the first-line treatment. With a median follow-up of 54 months, 12 patients experienced relapse or progression of the tumor. The 3- and 5-year disease-free survival (DFS) rates were 78.0%±6.5% and 75.0%±6.9%, respectively, and overall survival (OS) rates were 85.3%±5.6% and 76.8%±6.9%, respectively. The LCA subtype was associated with poorer DFS (P=0.023) and OS (P=0.008), compared with non-LCA subtypes.
The outcomes of children and adolescents with AvR MBL treated with radiation including CSI <2500 cGy and chemotherapy, are compatible to those in Western countries; however, the LCA subtype has a poor outcome with this strategy.
尽管颅脊髓照射（CSI） 2340cGy 加肿瘤加量化疗已被确立为西国家儿童平均风险（AvR）髓母细胞瘤（MBL）的标准治疗方法，但最近有一些关于韩国和其他亚洲儿童使用该策略治疗 AvR MBL 结果的报道。我们研究了在首尔国立大学儿童医院接受 CSI<2500cGy 和化疗治疗的韩国 AvR MBL 儿童的结果。
2001 年 1 月至 2010 年 12 月，分析了在首尔国立大学儿童医院接受手术治疗后接受包括 CSI<2500cGy 和化疗在内的放射治疗的 42 例 AvR MBL 患者的临床特征和结果。
患者的中位年龄为 9 岁（范围：3-18.8 岁），29 例为男性。组织学亚型为经典型 28 例，结节/促结缔组织增生型 7 例，大细胞/间变型（LCA）7 例。所有患者均接受辅助放疗（CSI 中位数 2340cGy 和加量）和多药化疗作为一线治疗。中位随访 54 个月后，12 例患者肿瘤复发或进展。3 年和 5 年无疾病生存率（DFS）分别为 78.0%±6.5%和 75.0%±6.9%，总生存率（OS）分别为 85.3%±5.6%和 76.8%±6.9%。与非 LCA 亚型相比，LCA 亚型与较差的 DFS（P=0.023）和 OS（P=0.008）相关。
接受 CSI<2500cGy 和化疗的儿童和青少年 AvR MBL 患者的结果与西方国家相似；然而，LCA 亚型的结果较差。
