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Intracranial malignant myopericytoma: case report and literature review
Jacques Marius Conradie1, Embre G Bonnet1, Anthony Thomas1
1Department of Neurosurgery, Robert Mangaliso Sobukwe Hospital, Kimberley, South Africa.
Abstract:
Malignant myopericytoma is an exceedingly rare soft tissue tumour with only 14 documented cases in the scientific literature. Myopericytomas can occur in association with HIV-AIDS and have a predilection for internal organs such as the bronchus, larynx, liver and brain. Ebstein-Barr virus encoded small RNA(EBER) was positive in three out of 14 cases. In this case report we present the second documented case of a malignant myopericytoma, with a positive EBER found intracranially - specifically in the supratentorial compartment - invading the superior sagittal sinus. Gross total excision of the tumour was achieved, and the patient responded well with no recurrence with only highly active antiretroviral therapy (HAART). No chemotherapy or radiotherapy has been necessary.
Insights
This case report details a rare malignant myopericytoma in the brain, positive for Ebstein-Barr virus encoded small RNA (EBER). Successful surgical removal and highly active antiretroviral therapy (HAART) led to no recurrence.
Area of Science:
- Oncology
- Virology
- Neuropathology
Background:
- Malignant myopericytoma is an extremely rare soft tissue neoplasm, with limited documented cases.
- These tumors can manifest in internal organs and have associations with Human Immunodeficiency Virus and Acquired Immunodeficiency Syndrome (HIV-AIDS).
- Ebstein-Barr virus encoded small RNA (EBER) has been detected in a subset of myopericytoma cases.
Observation:
- This report presents the second documented intracranial malignant myopericytoma.
- The tumor was located in the supratentorial compartment and invaded the superior sagittal sinus.
- The patient was co-infected with HIV-AIDS and tested positive for EBER.
Findings:
- Gross total excision of the intracranial malignant myopericytoma was successfully performed.
- The patient showed a positive response to highly active antiretroviral therapy (HAART).
- No recurrence was observed, and no chemotherapy or radiotherapy was required.
Implications:
- This case expands the understanding of malignant myopericytoma presentation and behavior.
- It highlights the potential role of EBER in specific myopericytoma cases.
- Effective management may involve surgical resection combined with HAART in HIV-positive patients, potentially avoiding aggressive treatments.
