Adult Medulloblastoma: Updates on Current Management and Future Perspectives.

SHH inhibitors SHH pathway chemotherapy medulloblastoma neurosurgery radiotherapy sonic hedgehog (SHH) sonidegib targeted therapy vismodegib

Journal

Cancers
ISSN: 2072-6694
Titre abrégé: Cancers (Basel)
Pays: Switzerland
ID NLM: 101526829

Informations de publication

Date de publication:
29 Jul 2022
Historique:
received: 12 06 2022
revised: 22 07 2022
accepted: 26 07 2022
entrez: 12 8 2022
pubmed: 13 8 2022
medline: 13 8 2022
Statut: epublish

Résumé

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.

Identifiants

pubmed: 35954372
pii: cancers14153708
doi: 10.3390/cancers14153708
pmc: PMC9367316
pii:
doi:

Types de publication

Journal Article Review

Langues

eng

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Auteurs

Enrico Franceschi (E)

Nervous System Medical Oncology Department, IRCCS Istituto delle Scienze Neurologiche di Bologna, Via Altura 3, 40139 Bologna, Italy.

Caterina Giannini (C)

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 59005, USA.
Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, 40126 Bologna, Italy.

Julia Furtner (J)

Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, 1090 Vienna, Austria.

Kristian W Pajtler (KW)

Hopp Children's Cancer Center Heidelberg (KiTZ), 69120 Heidelberg, Germany.
Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Consortium for Translational Cancer Research (DKTK), 69120 Heidelberg, Germany.
Department of Pediatric Oncology, Hematology and Immunology, Heidelberg University Hospital, 69120 Heidelberg, Germany.

Sofia Asioli (S)

Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, 40126 Bologna, Italy.
Pituitary Unit, IRCCS Istituto Delle Scienze Neurologiche Di Bologna, Via Altura 3, 40139 Bologna, Italy.

Raphael Guzman (R)

Department of Neurosurgery, University Hospital of Basel, 4031 Basel, Switzerland.

Clemens Seidel (C)

Department of Radiation Oncology, University Hospital Leipzig, 04103 Leipzig, Germany.

Lidia Gatto (L)

Department of Oncology, AUSL of Bologna, 40139 Bologna, Italy.

Peter Hau (P)

Wilhelm Sander NeuroOncology Unit & Department of Neurology, University Hospital Regensburg, 93055 Regensburg, Germany.

Classifications MeSH