Introduction
Medulloblastoma (MB) is the most common malignant brain tumor of childhood (1). The current multimodal therapy in children includes maximal safe resection, radiation therapy, and chemotherapy. In high-income countries (HIC), medulloblastoma survival outcomes are 80% for standard-risk and 60% for high-risk patients (2). These outcomes are largely due to proper risk stratification, surgical expertise, timely radiation therapy, advancements in imaging technology and supportive care, with current strategies focused on lessening the long-term sequelae of treatment (3–6). However, these results have not been replicated in patients living in low-and middle-income countries (LMIC), where almost 80% of children with cancer in the world live (7–9).
Patients with pediatric brain cancers in LMIC have lower survival rates for a multitude of reasons, such as barriers in accessing medical services, shortage of primary care professionals, unawareness of brain tumor presenting symptoms, difficulty in patient referral to complex health care settings, and a lack of availability of sub-specialty care and appropriate expertise (10–13). Data from predominantly HIC have found that delays in care and lack of specialized care can impact outcomes, specifically in pediatric brain tumors (14–16). The most common initial presenting symptoms (ie: headache, nausea, emesis) can simulate many benign conditions in childhood frequently leading to delays in diagnosis. Delays in imaging (due to lack of resources) can also affect staging and risk stratification contributing to under or overtreatment(16). These same factors that negatively impact outcomes are likely present, if not exacerbated, in LMIC.
Brazil is an upper middle-income country (UMIC) according to the World Bank classification in 2018 (17), with a gross national income per capita of US$ 9,140. With a population of more than 209 million inhabitants, Brazil is a country with vast socio-economical disparaties within its five major regions. The National Cancer Institute (INCA) is located in the state of Rio de Janeiro, in southeastern Brazil. INCA is the branch of the Ministry of Health responsible for leading a country-wide policy for prevention and cancer control in the country(18). It is a tertiary care referral center for the treament of adult and children with cancer free of charge within Brazil´s Unified Public Health System (SUS). INCA’s pediatric treatment approach is comprised of a multidisciplinary team of pediatric oncologists, neurosurgeons, neuro-radiologists, pathologists, radiation-oncologists, among others. Patients who are identifed to be high-risk for abandoning treatment receive additional support with food, lodging and transportation from the government and nonprofit organizations(19). On average there are 200 new solid tumor pediatric patients registered annually with 40-50 of them being patients with new brain tumors.
The aim of this study is to describe the epidemiological characteristics and provide survival outcome data of children treated for medulloblastoma at a single-institution, public, tertiary care center in Rio de Janeiro, Brazil.