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Supratentorial ectopic ependymoma
O Vernet1, J P Farmer, K Meagher-Villemure
1Department of Neurosurgery, Montreal Children's Hospital, McGill University, Quebec, Canada.
Insights
This study presents a rare case of an ectopic ependymoma in an 11-year-old female, found far from the brain's ventricles. This finding challenges typical ependymoma locations and prompts discussion on its origins.
Area of Science:
- Neuro-oncology
- Pediatric Neurosurgery
- Pathology
Background:
- Ependymomas are tumors typically originating from the ependymal cells lining the brain's ventricular system.
- Typical locations include the posterior fossa and supratentorial ventricles.
Observation:
- An 11-year-old female presented with symptoms including headache, nausea, vomiting, papilledema, and neurological deficits.
- Imaging revealed a 3 cm left precentral subcortical lesion, notably distant from the ventricular surface.
- Surgical resection confirmed a supratentorial ectopic ependymoma.
Findings:
- Histopathological, immunohistochemical, and electron microscopic analyses confirmed the diagnosis of ependymoma.
- The tumor was located in the centrum semiovale white matter, approximately 3 cm from the ventricle.
- Post-operative imaging and staging investigations ruled out tumor dissemination.
Implications:
- This case highlights the possibility of ependymomas occurring in extranventricular locations, challenging conventional understanding.
- Further research into the pathogenesis of ectopic ependymomas is warranted.
- Such rare presentations necessitate a broad differential diagnosis in pediatric neuro-oncology.
Background:
Ependymomas usually arise from the ventricular surface.
Methods:
We report an 11-year-old female who presented with a supratentorial ectopic ependymoma.
Results:
The patient presented with a two-month-history of progressive headache, nausea and vomiting. Examination revealed papilledema, horizontal nystagmus, diplopia on upward gaze, and right pronator drift. CT scan showed an enhancing left precentral subcortical lesion measuring 3 cm in diameter with associated edema and mass effect. Its medial border was located 3 cm from the ependymal surface of the ventricle. A firm tumour was dissected from the centrum semiovale white matter, and removed in toto as confirmed on MRI. Pathological examination revealed histological, immuno-histochemical and electron microscopic features consistent with an ependymoma. Spine MRI and bone marrow aspirate, as well as lumbar puncture of cytology failed to show any dissemination.
Conclusion:
From the literature review, this represents an exceptional ependymoma located at the distance from the ventricular system or cisterns. Different pathogenic alternatives are discussed.