Primary intracranial extraskeletal myxoid chondrosarcoma in the sphenoid sinus: A case report and literature review
Abstract
Background: Extraskeletal myxoid chondrosarcoma (EMC) is an exceedingly rare soft tissue sarcoma of low to intermediate grade. Its occurrence in the sphenoid sinus is exceedingly rare, and to our knowledge, no prior cases have been reported in this location.
Case Description: We report a case of a 37-year-old woman who presented with dizziness, weakness, and gait instability. Head computed tomography and magnetic resonance imaging revealed a soft tissue mass measuring approximately 25 mm × 20 mm × 12 mm in the left sphenoid sinus, with multiple linear and granular calcifications. The mass invaded the clivus and the left internal carotid artery canal. Postoperative histopathological and immunohistochemical findings confirmed the diagnosis of extraskeletal myxoid chondrosarcoma. After two months of follow-up, the patient recovered well with no tendency toward tumor recurrence.
Conclusion: To our knowledge, this is the first reported case of a primary intracranial extraskeletal myxoid chondrosarcoma arising in the sphenoid sinus. The distinctive imaging features—particularly the prominent calcification and the characteristic pattern of secondary bone invasion from an extra-axial origin—provide crucial diagnostic clues for this rare tumor.
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