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Infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: Two case reports
1Department of Neurosurgery Department of Pathology, West China Hospital of Sichuan University.
Rationale:
Intracranial medulloepitheliomas are extremely rare and highly malignant. Intraventricular medulloepitheliomas are even rarely reported, and little is known about the clinical features.
Patient Concerns:
In this article, we report two cases of intracranial medulloepitheliomas. In the first patient, a one-month old boy, the tumor was located in in right lateral ventricle, which was the first report of such location of this disease; in the second patient, an eleven-month old girl, the tumor was in right cerebellopontine angle.
Diagnoses:
Both patients were diagnosed as medulloepithelioma by pathologists.
Interventions:
Both patients underwent craniotomy to resect the lesion totally.
Outcomes:
The boy underwent chemotherapy after operation and was alive 3 months after operation. The girl died 6 months after operation, despite aggressive adjuvant chemotherapy.
Lessons:
Surgical resection is safe and effective to prolong patient survival. However, despite aggressive adjuvant therapy, prognosis of medulloepithelioma remains poor, and further study is needed to improve treatment of this rare disease.
Insights
Intracranial medulloepitheliomas are rare, malignant brain tumors. Surgical resection may prolong survival, but aggressive treatments offer poor prognosis, necessitating further research for better outcomes.
Area of Science:
- Neuro-oncology
- Pediatric Neurosurgery
Background:
- Intracranial medulloepitheliomas are exceptionally rare and highly malignant central nervous system tumors.
- Intraventricular medulloepitheliomas are seldom reported, with limited understanding of their clinical characteristics.
Observation:
- This report details two pediatric cases of intracranial medulloepithelioma.
- Case 1: A one-month-old boy with a tumor in the right lateral ventricle, a previously undocumented location.
- Case 2: An eleven-month-old girl with a tumor in the right cerebellopontine angle.
Findings:
- Both patients underwent complete surgical resection via craniotomy.
- The infant boy survived for 3 months post-operation with chemotherapy.
- The infant girl succumbed 6 months post-operation despite intensive adjuvant chemotherapy.
Implications:
- Complete surgical resection appears to be a safe and effective method for extending patient survival.
- The overall prognosis for medulloepithelioma remains poor, underscoring the urgent need for novel therapeutic strategies.
- Further research is critical to enhance treatment efficacy for this rare pediatric brain tumor.
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