Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Aug;6(4):100173.
doi: 10.1016/j.esmoop.2021.100173. Epub 2021 Jun 9.

How we treat medulloblastoma in adults

E Franceschi  1 , C Seidel  2 , F Sahm  3 , K W Pajtler  4 , P Hau  5
Affiliations
Review

How we treat medulloblastoma in adults

E Franceschi et al. ESMO Open. 2021 Aug.

Erratum in

Abstract

Medulloblastoma is a rare tumour in postpubertal patients and adults that is potentially curable. Several subgroups have been defined that are associated with clinical features, have different prognoses, and in some cases offer personalized treatment options. In adults, the sonic hedgehog (SHH) subtype is the most common subtype, followed by the wingless (WNT) and group 4 subtypes. Multimodal therapies allow 5-year overall survival rates of up to 70%. However, in adults, therapeutic evidence from prospective randomized trials is largely lacking. Therefore, regardless of individual risk, most patients are currently treated uniformly with craniospinal chemoradiation with a boost to the tumour bed, followed by maintenance chemotherapy, usually with alkylating agents. In Europe, the so-called Packer regimen, together with cisplatin-etoposide regimens, is the most commonly used chemotherapy option. Targeted treatment approaches have not yet been implemented, although tumour biology is well understood and offers personalized approaches, especially for the SHH subgroup. At relapse, rapid resistance occurs frequently, necessitating repositioning of these agents in an earlier treatment phase. Due to the good to intermediate prognosis, patients with medulloblastoma require structured long-term clinical follow-up including MRI of the brain, monitoring of side effects, and psychosocial and fertility counselling. Recently, clinical trials have been initiated with the aim of de-escalating treatment to reduce toxicity and adding targeted therapies to increase efficacy, with the main goal of therapy to cure the tumour while maintaining the physical and psychosocial integrity of affected patients. This article summarizes our opinion on the diagnosis and treatment of medulloblastoma in adults.

Keywords: adult; chemotherapy; classification; medulloblastoma; radiotherapy.

PubMed Disclaimer

Conflict of interest statement

Disclosure EF has received honoraria for lectures, consultation or advisory board participation from the following for-profit company: Karyopharm Therapeutics. CS has received honoraria for lectures, consultation or advisory board participation from the following for-profit companies: AbbVie, Bristol-Myers Squibb, HRA Pharma, Medac, Roche. FS has received honoraria (speaker) lectures, consultation or advisory board participation from the following for-profit companies: AbbVie, Bayer, Illumina. PH has received honoraria for lectures, consultation or advisory board participation from the following for-profit companies: Antisense Pharma, Bristol-Myers Squibb, GlaxoSmithKline, Lilly, Medac, Merck Sharp & Dome, Nanotherm, Novartis, Novocure, Roche. The following for-profit companies have supported clinical trials and contracted research conducted by PH with payments made to his institution: Antisense Pharma. KWP has declared no conflicts of interest.

Figures

Figure 1
Figure 1
Diagnostic and therapeutic pathway in adult medulloblastoma (first-line therapy situation). GTR, gross total resection; MB, medulloblastoma; MRI, magnetic resonance imaging; SHH, sonic hedgehog; WHO, World Health Organization; WNT, wingless.
See this image and copyright information in PMC

References

    1. Dirven L., Luerding R., Beier D. Neurocognitive functioning and health-related quality of life in adult medulloblastoma patients: long-term outcomes of the NOA-07 study. J Neurooncol. 2020;148(1):117–130. - PMC - PubMed
    1. Warren K.E., Vezina G., Poussaint T.Y. Response assessment in medulloblastoma and leptomeningeal seeding tumors: recommendations from the Response Assessment in Pediatric Neuro-Oncology committee. Neuro Oncol. 2018;20(1):13–23. - PMC - PubMed
    1. Padovani L., Sunyach M.P., Perol D. Common strategy for adult and pediatric medulloblastoma: a multicenter series of 253 adults. Int J Radiat Oncol Biol Phys. 2007;68(2):433–440. - PubMed
    1. Brandes A.A., Franceschi E., Tosoni A., Blatt V., Ermani M. Long-term results of a prospective study on the treatment of medulloblastoma in adults. Cancer. 2007;110(9):2035–2041. - PubMed
    1. Thompson E.M., Hielscher T., Bouffet E. Prognostic value of medulloblastoma extent of resection after accounting for molecular subgroup: a retrospective integrated clinical and molecular analysis. Lancet Oncol. 2016;17(4):484–495. - PMC - PubMed

Substances