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Intrinsic epidermoid cyst of the brainstem in children-review and case report
Adib Saráty Malveira1, Marcos Devanir Silva da Costa2, Esdras Ismael Borrayo Flores2
1Department of Neurology and Neurosurgery, Universidade Federal de Sao Paulo, Rua Napoleão de Barros, 715, 6th Floor, Zip Code: 04024-002, Sao Paulo, SP, Brazil. adibsaraty@hotmail.com.
Insights
Intrinsic brainstem epidermoid cysts are rare pediatric tumors. Maximum surgical resection is key for functional preservation in these young patients.
Area of Science:
- Neuroscience
- Pediatric Neurosurgery
- Oncology
Background:
- Intrinsic brainstem epidermoid cysts are exceptionally rare congenital tumors.
- These lesions present unique diagnostic and therapeutic challenges due to their location.
Observation:
- A systematic review of pediatric brainstem epidermoid cysts was conducted.
- Twenty-one cases were identified, with a mean patient age of 4.85 years.
- Common symptoms included cranial nerve deficits and pyramidal tract signs.
Findings:
- Cranial nerve VII palsy was the most frequent neurological deficit observed.
- Gross total resection was the primary treatment modality in the presented case.
- The study highlights the specific characteristics and clinical presentation of these rare tumors.
Implications:
- Optimal treatment involves maximal safe resection to preserve neurological function.
- Early diagnosis and surgical intervention are crucial for favorable outcomes.
- Further research into the specific molecular mechanisms and long-term outcomes is warranted.
Purpose:
The study aimed to summarize all published cases of intrinsic brainstem epidermoid cysts in a timeline to highlight the specific characteristics and individualize the disease, in addition to discussing the best treatment used.
Methods:
The scientific literature on pediatric cases of intrinsic epidermoid cysts of the brainstem was analyzed. We present the case of a 1.5-year-old male with incidental presentation, who was treated with gross total resection. We summarize all previously published cases to individualize the disease.
Results:
We identified 21 patients, including 10 boys and 11 girls, with a mean age of 4.85 (1-15) years at the time of surgery. The most frequent symptoms were cranial nerve palsy (71.4%), pyramidal tract deficit (57.14%), and headache (52.38%). Among the affected cranial nerves, VII was the most frequently reported in 10 patients.
Conclusion:
Brainstem epidermoid cysts are extremely rare pathologies with relevant age involvement in young children. The treatment objective should be the maximum resection of the lesion through a careful approach and with the appropriate tools for the functional preservation of the patient.
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