Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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August 1, 2024
Chordoid Meningioma
- Background:
- Chordoid meningioma is a rare histologic variant classified as a WHO grade 2 tumor and is prone to a high rate of recurrence following subtotal resection.
- Clinical Presentation:
- Neurologic manifestations depend on tumor size, location, and associated mass effect. Patients may present with seizures, headaches, visual disturbances, and cognitive changes, among other manifestations.
- Key Diagnostic Features:
- Unlike other meningiomas that are frequently associated with restricted diffusion, chordoid meningiomas show facilitated diffusion and low attenuation on CT.
- ADC > 1.39 and normalized ADC > 1.93 (normalized to normal contralateral white matter) are highly indicative of a chordoid meningioma.
- Differential Diagnosis:
- Other meningioma subtypes: usually associated with restricted diffusion. Both chordoid meningioma and other subtypes can present with variable degrees of edema and calcification.
- Dural metastases: can be indistinguishable from meningioma but tend to present with restricted diffusion due to high cellularity.
- Lymphoma: dural lymphoma is usually secondary and seen in patients with systemic disease. Primary dural lymphoma is rare. Lymphoma typically shows very high restricted diffusion due to high cellularity.
- Solitary fibrous tumors: are often indistinguishable from meningiomas. On a T2-weighted MRI, these may show a unique "yin-yang" pattern, comprising areas of both low and high signal intensity. Additionally, the presence of dural tails is less frequent in solitary fibrous tumors as compared with meningiomas.
- In general, the presence of a true dural tail (enhancing brighter than the tumor and tapering peripherally) favors meningioma over other etiologies.
- Treatment:
- Like other meningiomas, the standard treatment typically involves a combination of surgical resection and radiotherapy, depending on individual factors such as tumor size, location, and recurrent or residual disease.