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.