Updates on Management of Adult Medulloblastoma

Abstract

Medulloblastoma (MB) is a malignant embryonal tumor of the posterior fossa and is the most common type of brain cancer in pediatric patients. In contrast, adult MB is very rare with an incidence of 0.6 per million per year and mostly affects young adults below the age of 40. Recent molecular analyses of pediatric and adult MB have classified these tumors into at least four individual molecular subgroups (SHH, WNT, group 3, and group 4) with distinct demographics, histology, and prognosis. The discrete biological composition of these tumors likely explains the marked heterogeneity in responses seen to conventional therapies such as radiation and cytotoxic chemotherapies. Given the low incidence of adult MB, prospective studies are challenging and scarce, and management guidelines are largely derived from the pediatric MB patient population and retrospective data. However, adult MB is clinically and molecularly distinct from pediatric MB and a comprehensive review of published literature on adult MB highlighting their differences is warranted. Here, we review the management of adult MB focusing on recent studies exploring the effectiveness of upfront chemotherapy, clinical trials in the context of molecular subgroup-specific therapies, and the potential role of immunotherapy in treating this disease.

Keywords: Adult medulloblastoma; Chemotherapy for treatment of medulloblastoma; Craniospinal radiation; Management algorithm for medulloblastoma; Medulloblastoma staging systems; Molecular subtypes of medulloblastoma.