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Dysplastic cerebellar gangliocytoma: a six-decade study
Emily K Chapman1, Tirone Young1, Zerubabbel Asfaw1
1Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
Journal of Neurosurgical Sciences
|October 28, 2025
Summary
Dysplastic cerebellar gangliocytoma (DCG) is a rare PTEN-associated tumor. MRI aids diagnosis, and surgical resection offers favorable outcomes, with ongoing research into targeted therapies.
Area of Science:
- Neuro-oncology
- Genetics
- Pathology
Background:
- Dysplastic cerebellar gangliocytoma (DCG) is a rare glioneuronal and neuronal tumor.
- Alterations in the phosphatase and tensin homolog (PTEN) gene are key in DCG pathogenesis.
- Understanding DCG trends and institutional cases is crucial for improved management.
Purpose of the Study:
- To analyze diagnostic and outcome trends of DCG over six decades.
- To present institutional cases of DCG.
- To investigate the association between DCG, PTEN mutations, and Cowden Syndrome.
Main Methods:
- Comprehensive literature review of online databases (1970-2024).
- Analysis of institutional pathology database for DCG cases (2000-2024).
- Dichotomization of data into adult and pediatric cohorts.
Main Results:
- 173 DCG cases (170 literature, 3 institutional) were analyzed.
- DCG predominantly affects adults (82%) and women (59%).
- Common symptoms include balance/coordination deficit; MRI shows a "tiger-stripe" pattern in 67%; 48.3% have PTEN mutations; 67% are associated with Cowden Syndrome.
Conclusions:
- DCG is a rare tumor linked to PTEN mutations, with MRI aiding diagnosis.
- Surgical resection is the primary treatment, leading to favorable outcomes.
- Further research into genetic screening and targeted therapies is recommended for refined diagnosis and management.
