Medulloblastoma (MB) is a malignant embryonal tumor of the posterior fossa belonging to
the family of primitive neuro-ectodermic tumors (PNET). MB generally occurs in pediatric age, but in
14–30% of cases, it affects the adults, mostly below the age of 40, with an incidence of 0.6 per million
per year, representing about 0.4–1% of tumors of the nervous system in adults. Unlike pediatric MB,
robust prospective trials are scarce for the post-puberal population, due to the low incidence of MB in
adolescent and young adults. Thus, current MB treatments for older patients are largely extrapolated
from the pediatric experience, but the transferability and applicability of these paradigms to adults
remain an open question. Adult MB is distinct from MB in children from a molecular and clinical
perspective. Here, we review the management of adult MB, reporting the recent published literature
focusing on the effectiveness of upfront chemotherapy, the development of targeted therapies, and
the potential role of a reduced dose of radiotherapy in treating this disease.
| Identifer | oai:union.ndltd.org:DRESDEN/oai:qucosa:de:qucosa:87875 |
| Date | 02 November 2023 |
| Creators | Franceschi, Enrico, Giannini, Caterina, Furtner, Julia, Pajtler, Kristian W., Asioli, Sofia, Guzman, Raphael, Seidel, Clemens, Gatto, Lidia, Hau, Peter |
| Publisher | MDPI |
| Source Sets | Hochschulschriftenserver (HSSS) der SLUB Dresden |
| Language | English |
| Detected Language | English |
| Type | info:eu-repo/semantics/publishedVersion, doc-type:article, info:eu-repo/semantics/article, doc-type:Text |
| Rights | info:eu-repo/semantics/openAccess |
| Relation | 3708, 10.3390/cancers14153708 |
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