Synonyms

Cavernoma; Cavernous hemangioma; Cavernous venous malformation; Cerebral cavernous malformation (CCM)

Definition

Cavernous angiomas are benign vascular malformations found within the CNS that may occur sporadically or in a familial pattern. Cavernous angiomas are also a complication of radiation therapy, especially in children. These angiomas can remain stable, enlarge over time, or bleed, and there are no factors that predict their occurrence or behavior.

Three genetic loci responsible for familial cavernous angioma (CCM1, CCM2, and CCM3) have been identified. Cavernomas are typically found supratentorially (approximately 80%), predominantly in the subcortical rolandic and temporal areas. Infratentorially, cavernous angiomas are most commonly found in the pons and cerebellar hemispheres (Sage and Blumbergs 2001). Originally thought to be relatively rare and most commonly detected during autopsy, the advent of MRI has led to an increased detection, with incidence rates now estimated between 0.02% and 0.8% of the general population. The size of the well-circumscribed, “mulberry-like” mass can range from less than 1 cm to greater than 4 cm. Prevalence rates are relatively equivalent among males and females. While it can remain asymptomatic lifelong, symptomatic presentation is most commonly seen in the third and fourth decades of life. However, newly symptomatic cases have been well reported throughout the life span. Women are more likely to present with hemorrhage and neurologic deficits (Del Curling et al. 1991).

Clinical manifestations, when present, vary significantly and generally correlate to location of the lesion. Most commonly reported symptoms include headache (6–65%), seizure (23–52%), focal neurological deficit (20–45%), and intracranial hemorrhage (13–25%) (Conway and Rigamonti 2006). Despite the regional affinity for frontal and temporal regions, no studies have specifically examined for selective neuropsychological deficits. Treatment can include observation, surgical resection, or stereotactic radiosurgery.