Management of high grade primary cerebellar tumours

Cerebellar high-grade gliomas (cHGG) are uncommon in adults, making up only about 1% of all high-grade gliomas. These tumours differ from supratentorial high-grade gliomas (sHGG) in terms of epidemiology, molecular traits, and the age of the patients. cHGG patients are typically younger and show a higher frequency of neurofibromatosis 1 (NF1) mutations, atypical RAS mutations, and H3K27M mutations. Standard treatment includes surgical resection followed by chemotherapy and radiation. Recent studies emphasize the genetic differences between cerebellar and supratentorial tumours, with new treatments targetting specific molecular abnormalities. Immunotherapy has shown limited effectiveness due to the unique tumour environment in cHGG, and further research is required to improve treatment strategies for these rare tumours.

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Publication Details

Journal
Journal of the Pakistan Medical Association
Published
2024-10-16
DOI
https://doi.org/10.47391/jpma.24-92
Primary Topic
Glioma Diagnosis and Treatment
Type
article
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article

Management of high grade primary cerebellar tumours

Muhammad Shahzad Shamim, Zanib Javed, Raheema Sadiq
Journal of the Pakistan Medical Association
Glioma Diagnosis and Treatment
article

Management of high grade primary cerebellar tumours

Muhammad Shahzad Shamim, Zanib Javed, Raheema Sadiq
article en

Abstract

Cerebellar high-grade gliomas (cHGG) are uncommon in adults, making up only about 1% of all high-grade gliomas. These tumours differ from supratentorial high-grade gliomas (sHGG) in terms of epidemiology, molecular traits, and the age of the patients. cHGG patients are typically younger and show a higher frequency of neurofibromatosis 1 (NF1) mutations, atypical RAS mutations, and H3K27M mutations. Standard treatment includes surgical resection followed by chemotherapy and radiation. Recent studies emphasize the genetic differences between cerebellar and supratentorial tumours, with new treatments targetting specific molecular abnormalities. Immunotherapy has shown limited effectiveness due to the unique tumour environment in cHGG, and further research is required to improve treatment strategies for these rare tumours.

Journal of the Pakistan Medical AssociationVol. 74(11)
Aga Khan University Hospital (PK)
Good health and well-being
Openalex Percentile: Top 69%
Glioma Diagnosis and Treatment
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