De novo giant cavernous malformation

Case contributed by Brian Alan Perry, II

Presentation

Patient presents with 3-4 week history of headache and nausea. The patient had a right MCA territory stroke 3 years prior. Workup revealed positive anticardiolipin antibody. The patient was also on estradiol-containing birth control at the time of the CVA.

Patient Data

Age: 20 years
Gender: Female

Large macrolobulated heterogeneous high attenuation lesion in the anterior left frontal lobe, with mass effect and moderate surrounding edema.  

Lobular anterior left frontal lobe hemorrhagic mass, with marked heterogeneity, internal loculation, and blood product of varying ages. Significant intrinsic T1 shortening, without apparent enhancement.  Classic "popcorn" appearance on T2-weighted imaging, with findings suggestive of cerebral cavernous malformation.  The mass measured up to 5.9 cm (anteroposterior). 

Small developmental venous anomaly noted along the anterior margin of the mass, draining to the superior sagittal sinus via a cortical vein (DVA more clearly elucidated with benefit of previous MRI).

No additional areas of abnormal susceptibility artifact.  

Incidental note of scattered foci of encephalomalacia in the right MCA territory.

MRI

28 months prior

No evidence of cavernous malformation on T2 or SWI. Small developmental venous anomaly noted in high anterior left fontal lobe, draining to the superior sagittal sinus via a cortical vein.  

Case Discussion

This is a case of de novo formation of a large cerebral cavernous malformation, in the setting of existing adjacent developmental venous anomaly and thrombotic disorder.  The lesion develops within a short window (under 2.5 years), suggesting relatively rapid growth via repeated hemorrhage.  

Cavernous malformations are traditionally considered to be congenital lesions, though de novo lesions have now been described in familial cases and in the setting of existing DVA.  The association between cavernous malformations and DVA's is well-described in the literature.  

It has been proposed that cavernous malformations that are coupled with a DVA are acquired lesions, which develop secondary to the corresponding altered flow dynamics and venous drainage.  In this case, there are likely patient factors (ACA positivity and exogenous estrogen) that facilitated formation and/or rapid growth of this low-flow vascular malformation.

Surgical resection performed, with pathology consistent with imaging diagnosis of cavernous malformation.

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Case information

rID: 66120
Published: 13th Feb 2019
Last edited: 10th Jul 2019
Inclusion in quiz mode: Included
Institution: Duke University Medical Center

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