Craniotomy for a Large and Aggressive De Novo Cavernous Malformation Resection in the Basal Ganglia Region.

BACKGROUND

Cerebral cavernous malformations (CCMs) are present in up to 0.5% of the general population. Although CCMs have been considered congenital lesions, numerous reports have observed de novo formations in patients with the familial form of CCM and in patients after cranial radiotherapy. Outside of these circumstances, there is scant evidence as to the potential etiologies of CCM.

METHODS

We present a 48-year-old woman with a medical history of endometrial hyperplasia concomitant endometrial polyps demonstrating a large de novo CCM, which grew to a large size in a period of 20 months. A previous magnetic resonance imaging scan showed no abnormalities. This CCM exhibited aggressive biological behavior characterized by recurrent overt bleeding and seizure. Histopathologic analysis confirmed the diagnosis of CCM. Here, we discuss the growth mechanisms of these lesions.

CONCLUSIONS

Given the patient's medical history and imaging findings, we propose that de novo CCMs can arise directly from angiogenic proliferation, secondary to BCL-2 overexpression from underlying causes. We hypothesize that inappropriate secretion of estrogen could have set off a genetic cascade with attendant endothelial proliferation. Thus, female hormones may play an important role in influencing the biological behavior of CCMs. The relationship between estrogen and CCM needs further investigation.