Pediatric Oncology Department, Instituto Nacional de Enfermedades Neoplásica, Lima, Perú.
Pediatrics Department, Nationwide Children's Hospital, Columbus, Ohio, USA.
Pediatr Blood Cancer. 2022 Oct;69(10):e29770. doi: 10.1002/pbc.29770. Epub 2022 May 20.
Medulloblastoma is the most common malignant brain tumor in children. While survival has improved in high-income countries (HIC), the outcomes for patients in low-to-middle-income countries (LMIC) are unclear. Therefore, we sought to determine the survival of children with medulloblastoma at the Instituto Nacional de Enfermedades Neoplasicas (INEN) between 1997 and 2013 in Peru.
Between 1997 and 2013, data from 103 children older than 3 years with medulloblastoma were analyzed. Fourteen patients were excluded. The patients were split into two distinct cohorts, 1997-2008 and 2009-2013, corresponding with chemotherapy regimen changes. Event-free (EFS) and overall survival (OS) were calculated using the Kaplan-Meier method, whereas prognostic factors were determined by univariate analysis (log-rank test).
Eighty-nine patients were included; median age was 8.1 years (range: 3-13.9 years). The 5-year OS was 62% (95% CI: 53%-74%), while EFS was 57% (95% CI: 48%-69%). The variables adversely affecting survival were anaplastic histology (compared to desmoplastic; OS: HR = 3.4, p = .03), metastasis (OS: HR = 3.5, p = .01; EFS: HR = 4.3, p = .004), delay in radiation therapy of 31-60 days (compared to ≤30 days; EFS: HR = 2.1, p = .04), and treatment 2009-2013 cohort (OS: HR = 2.2, p = .02; EFS: HR = 2.0, p = .03).
Outcomes for medulloblastoma at INEN were low compared with HIC. Anaplastic subtype, metastasis at diagnosis, delay in radiation therapy, and treatment in the period 2009-2013 negatively affected the outcomes in our study. Multidisciplinary teamwork, timely delivery of treatment, and partnerships with loco-regional groups and colleagues in HIC is likely beneficial.
髓母细胞瘤是儿童中最常见的恶性脑肿瘤。虽然高收入国家（HIC）的生存率有所提高，但中低收入国家（LMIC）患者的预后尚不清楚。因此，我们试图确定秘鲁 INEN （Instituto Nacional de Enfermedades Neoplasicas）在 1997 年至 2013 年间患有髓母细胞瘤的儿童的生存率。
在 1997 年至 2013 年间，分析了 103 名年龄大于 3 岁的患有髓母细胞瘤的儿童的数据。排除了 14 名患者。将患者分为两个不同的队列，1997-2008 年和 2009-2013 年，与化疗方案的变化相对应。采用 Kaplan-Meier 方法计算无事件生存率（EFS）和总生存率（OS），并通过单因素分析（log-rank 检验）确定预后因素。
纳入 89 例患者；中位年龄为 8.1 岁（范围：3-13.9 岁）。5 年 OS 为 62%（95%CI：53%-74%），EFS 为 57%（95%CI：48%-69%）。影响生存的变量包括间变组织学（与促结缔组织增生型相比；OS：HR=3.4，p=0.03）、转移（OS：HR=3.5，p=0.01；EFS：HR=4.3，p=0.004）、放疗延迟 31-60 天（与≤30 天相比；EFS：HR=2.1，p=0.04）和 2009-2013 年治疗队列（OS：HR=2.2，p=0.02；EFS：HR=2.0，p=0.03）。
与 HIC 相比，INEN 髓母细胞瘤的治疗结果较低。间变亚型、诊断时转移、放疗延迟以及 2009-2013 年期间的治疗对本研究的结果产生了负面影响。多学科团队合作、及时治疗以及与当地团体和 HIC 同事的合作可能是有益的。
