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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April 2, 2020
Giant Cavernous Sinus Hemangioma
- Background:
- Intracranial giant cavernous malformation is a rare, benign condition with a female predominance. It accounts for less than 1% of parasellar masses and can occur in any age group.
- Clinical Presentation:
- Symptoms, such as headache, visual disturbances, and cranial nerve palsies, are usually related to mass effect.
- Key Diagnostic Features:
- Typical parasellar location in the middle cranial fossa
- Noninvasive behavior displacing the adjacent structures
- Well-circumscribed, hyperdense mass with sparse calcification on CT scans
- Low intensity to isointensity on T1WI, with marked hyperintense signal on T2WI, scattered magnetic susceptibility artifacts in SWI (hemosiderin and calcium deposits), and no restriction on DWI/ADC map
- Peripheral, discontinuous, and nodular enhancement, which progresses centripetally on delayed images, similar to the typical liver hemangiomas
- Differential Diagnoses:
- Invasive pituitary macroadenoma: The pituitary gland is hard to distinguish from the mass. With pituitary fossa enlargement the enhancement pattern can be homogeneous or heterogeneous depending on the cystic degeneration/hemorrhagic component. The marked T2WI hyperintensity areas in an adenoma should not enhance, as they represent cystic degeneration.
- Meningioma: The enhancement pattern is usually intense and homogeneous on early phases (not progressive as in cavernous hemangiomas), and can present a dural tail. Its fibrous component may determine low signal in T2WI and hyperostosis can be present. Larger meningiomas can extend through the foramen ovale.
- Schwannoma: Soft, well-marginated and lobulated tumor that shapes itself to the structures/foramina, expanding them. It is usually hyperintense in T2WI and has an avid homogeneous enhancement. Larger schwannomas can be heterogeneous in T2WI due to sparse areas of cystic degeneration and, therefore, present heterogeneous enhancement. Rare magnetic susceptibility artifacts in SWI may be present. The most common in this location is the trigeminal schwannoma in Meckel's cave and it can extend to the cistern portion of the nerve.
- Chondrosarcoma: Off-midline malignancy with high T2 signal, scattered chondroid calcifications (ringlike), and mild heterogeneous enhancement pattern, whose typical location is centered on the petro-occipital fissure
- Treatment:
- Cavernous angiomas have a strong tendency to bleed profusely during surgery or biopsy; therefore, this diagnosis must be considered so that there is better surgical planning with better outcomes.
- Other possibilities, such as preoperative radiation therapy and gamma knife surgery, have been described.
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