Pediatric medulloblastoma in the molecular era: what are the surgical implications?

Abstract

Pediatric brain tumors are the leading cause of childhood cancer mortality with medulloblastoma (MB) representing the most frequent malignant tumor. Although standardization of therapy resulted in a 2-fold reduction in mortality in patients with MB by 2002, it became clear that further improvements in clinical outcome would require a deeper understanding of the biology of MB. Employing the four main molecular MB subgroups (Wnt, Shh, Group 3 and Group 4), a restratification into clinicogenomic risk categories quantified an unacceptable survival for the high-risk group, urging researchers to focus their efforts towards acquiring a greater biological understanding of these children. Advancing in parallel with the molecular characterization and understanding of pediatric MB is the clinicogenomic correlations giving rise to recommendations for neurosurgical care. While unique observations that distinct radiological patterns can be identified to inform the MB molecular subgroup preoperatively, current neurosurgical practice remains maximal safe surgical resection followed by risk-adapted provision of adjuvant therapy in the context of a clinical trial.