A rare case of primary intracranial myxoma in a 10-year-old male: Case report and literature review

Abstract

within the central nervous system, distinct from the more common cardiac myxoma with central nervous system embolization. We report the case of a previously healthy 10-year-old male who presented with a first-time witnessed generalized tonic-clonic seizure lasting approximately 90 seconds, with clinical features suggesting a focal onset (head version to the left and asymmetric tonic posturing of the right arm). Postictally, the patient regained full consciousness after 15 minutes with no focal neurological deficits. Neuroimaging revealed an extra-axial, dura-based lesion in the left frontal lobe. Preoperative magnetic resonance imaging (MRI) demonstrated a well-defined, T1-hypointense, T2/FLAIR-hyperintense lesion with strong homogeneous contrast enhancement, insinuating between the superior and middle frontal gyri without significant mass effect. The patient underwent gross total resection via a pterional approach, revealing a well-capsulated, gelatinous, multilobulated tumor. Histopathological examination confirmed a connective tissue lineage neoplasm with stellate cells embedded in a myxoid matrix, consistent with a myxoma. Post-operative echocardiography and cardiac MRI ruled out a cardiac primary source, confirming the diagnosis of PIM. The patient was initiated on levetiracetam 20 mg/kg/day postoperatively, which was successfully tapered and discontinued after 3 months. The patient had an uneventful recovery with no seizure recurrence at 18-month follow-up. This case underscores PIM as a diagnostic consideration for dura-based, well-circumscribed intracranial lesions in children. A multimodal approach combining characteristic neuroimaging, meticulous histopathology, and thorough systemic investigation to exclude a cardiac source is essential for accurate diagnosis and management, which yields an excellent prognosis following complete surgical resection.