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
. 2024 Dec 20;16(24):4242.
doi: 10.3390/cancers16244242.

Adult Cerebellopontine Angle Medulloblastoma: A Systematic Review of Clinical Features, Management Approaches, and Patient Outcomes

Kishore Balasubramanian  1   2 , Abdurrahman F Kharbat  2 , Francisco Call-Orellana  3 , Sherwin A Tavakol  2 , Grace R Fassina  2 , Christopher Janssen  1 , Othman Bin Alamer  4 , Jeffrey A Zuccato  2 , Ian F Dunn  2
Affiliations
Review

Adult Cerebellopontine Angle Medulloblastoma: A Systematic Review of Clinical Features, Management Approaches, and Patient Outcomes

Kishore Balasubramanian et al. Cancers (Basel). .

Abstract

Objective: The aim of this study was to systematically review the existing individual patient data in the literature on adult cerebellopontine angle (CPA) medulloblastoma (MB) and characterize the patient presentation, management strategies used, and oncological outcomes of this rare entity to guide future clinical practice.

Methods: Following PRISMA guidelines, a systematic review was conducted by searching PubMed, EMBASE, Web of Science, and Cochrane databases from inception to 19 June 2024. Studies regarding adult patients with histologically confirmed MB radiographically confirmed to be located in the CPA were included. Clinical data were synthesized, and predictors of outcomes were evaluated.

Results: Twenty-seven studies with 42 adult CPAMB patients were included. The median age was 32 years (range: 19-56). Headaches (81%), cranial neuropathy (90%), cerebellar dysfunction (79%), and nausea/vomiting (50%) were typical presenting features. The predominant histological subtype was the classic variant. Maximal safe surgical resection was performed, most commonly using a retrosigmoid approach, and 60% of cases received a gross total resection. Most patients received adjuvant treatment (93%), typically chemoradiotherapy. The recurrence rate was 11% after a median of 18 months of follow-up. Relatively high survival rates of 96%, 85%, and 85% were observed at 1, 3, and 5 years, respectively. Patients who received adjuvant therapy had significantly better recurrence and greater overall survival outcomes.

Conclusions: These results support the consideration of MB in young adult patients presenting with CPA tumors with radiographical features suggestive of hypercellularity and the utilization of a management strategy of maximal safe resection plus post-operative craniospinal irradiation along with chemotherapy to optimally treat these rare patients.

Keywords: adjuvant therapy; brain tumor; central nervous system malignancy; cerebellopontine angle; medulloblastoma; neuro-oncology; posterior fossa mass; retrosigmoid craniotomy; translabyrinthine approach.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
PRISMA study inclusion flowchart: this flowchart illustrates the number of studies originally identified, study exclusions, and the final inclusion of 27 studies for the review.
Figure 2
Figure 2
Single-arm Kaplan–Meier survival plot for overall survival: This figure portrays the unadjusted Kaplan–Meier plot showing overall survival.
Figure 3
Figure 3
Single-arm Kaplan–Meier survival plot for recurrence-free survival: This figure portrays the unadjusted Kaplan–Meier plot showing recurrence-free survival.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Giordana M.T., Schiffer P., Lanotte M., Girardi P., Chio A. Epidemiology of adult medulloblastoma. Int. J. Cancer. 1999;80:689–692. doi: 10.1002/(SICI)1097-0215(19990301)80:5<689::AID-IJC10>3.0.CO;2-G. - DOI - PubMed
    1. Wu T., Qu P.R., Zhang S., Li S.W., Zhang J., Wang B., Liu P., Li C.D., Zhao F. The clinical treatment and outcome of cerebellopontine angle medulloblastoma: A retrospective study of 15 cases. Sci. Rep. 2020;10:9769. doi: 10.1038/s41598-020-66585-7. - DOI - PMC - PubMed
    1. Aqel W., Salman A., Aldarawish A., Bakri I. Medulloblastoma at the cerebello-pontine angle resembling vestibular schwannoma: A case report and review of the literature. Int. J. Surg. Case Rep. 2022;99:107695. doi: 10.1016/j.ijscr.2022.107695. - DOI - PMC - PubMed
    1. Griepp D.W., Miller A., Klein J., Chaudhri A.A., Moawad S., Rehmani R., Rahme R. Dural-Based Posterior Fossa Medulloblastoma Mimicking a Petrous Meningioma in Late Adulthood. Ochsner J. 2022;22:176–181. doi: 10.31486/toj.21.0064. - DOI - PMC - PubMed
    1. Menon G., Krishnakumar K., Nair S. Adult medulloblastoma: Clinical profile and treatment results of 18 patients. J. Clin. Neurosci. 2008;15:122–126. doi: 10.1016/j.jocn.2007.06.007. - DOI - PubMed

LinkOut - more resources