Primary intracranial myxoma mimicking a frontal parasagittal meningioma: A Case Report and Review of the Literature

Primary intracranial myxomas are exceptionally rare benign mesenchymal tumors, most commonly involving the skull base, whereas supratentorial frontal parasagittal extra-axial localizations remain exceedingly uncommon and may radiologically mimic meningiomas, making preoperative diagnosis particularly challenging. We report the case of a 58-year-old patient presenting with chronic fronto-orbital headaches complicated by a generalized tonic–clonic seizure. Brain magnetic resonance imaging revealed a well-circumscribed multilobulated right frontal parasagittal extra-axial lesion, hypointense on T1-weighted images, hyperintense on T2/FLAIR sequences, and showing marked heterogeneous gadolinium enhancement, initially suggestive of meningioma or another mesenchymal tumor. Given the symptomatic presentation, the patient underwent gross-total surgical resection through a right frontolateral craniotomy. Histopathological examination demonstrated a hypocellular myxoid tumor composed of spindle-shaped cells embedded within an abundant myxoid stroma, with immunohistochemical positivity for vimentin and S-100 protein, negativity for epithelial membrane antigen, and a low proliferative index (Ki-67 <1%), confirming the diagnosis of primary intracranial myxoma. Postoperative cardiac investigations excluded an underlying cardiac myxoma, supporting a primary intracranial origin. The postoperative course was uneventful, and follow-up magnetic resonance imaging showed no recurrence. Primary intracranial myxomas should be considered in the differential diagnosis of atypical extra-axial frontal lesions because radiological findings remain non-specific, and definitive diagnosis relies on histopathological and immunohistochemical confirmation. Gross-total resection remains the cornerstone of treatment and the principal prognostic factor for long-term disease control.