Abstract
ABSTRACTObjective: To describe the clinical, demographic, anatomopathological, molecular, and survival characteristics of patients with medulloblastoma.Methods: Retrospective study based on patient information obtained from the review of medical records. Overall and event-free survival were analyzed using the Kaplan-Meier estimator, and the curves were compared by the log-rank test.Results: Among the patients investigated, 70 were male (66%), and age at diagnosis ranged from 2 months to 22 years. The most frequent signs and symptoms were headache (80.8%) and vomiting (75.8%). Regarding treatment, most patients (63.2%) underwent complete surgical resection, with a predominance of classic histology (63.2%). The 5-year overall survival rate was 67.9%, and the 10-year rate was 64.2%. Patients with molecular profile characteristic of the wingless (WNT) subgroup had a better prognosis, with 5-year overall survival of 75%.Conclusions: The clinical, demographic, anatomopathological, and molecular characteristics of patients with medulloblastoma described in the present study were mostly similar to those reported in the literature. Patients submitted to complete tumor resection had better clinical outcomes than those who underwent incomplete resection/biopsy. Patients classified as high-risk showed worse overall and event-free survival than those in the standard-risk group, and the presence of metastasis at diagnosis was associated with recurrence.
Highlights
Central nervous system (CNS) neoplasms are the second most frequent type of cancer reported in children and adolescents.[1,2] CNS tumors represent the main cause of cancer-related mortality
Medulloblastoma, a primitive neuroectodermal tumor originated in the cerebellum, is one of the most common malignant brain tumors in children, representing 20% of CNS tumors
The present study analyzed 106 cases of medulloblastoma treated in a single health facility specialized in pediatric hematology-oncology in the city of Campinas, São Paulo
Summary
Central nervous system (CNS) neoplasms are the second most frequent type of cancer reported in children and adolescents.[1,2] CNS tumors represent the main cause of cancer-related mortality. The sequelae caused by tumors and their treatments result in high morbidity among children.[3,4,5]. Medulloblastoma, a primitive neuroectodermal tumor originated in the cerebellum, is one of the most common malignant brain tumors in children, representing 20% of CNS tumors. It is an invasive tumor, classified as grade IV by the World Health Organization (WHO),[6] whose prognosis is unfavorable to children under three years of age. The incidence is low, ranging from 0.5 to 1%.7
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