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Cavernous angiomas: deconstructing a neurosurgical disease

JNSPG 75th Anniversary Invited Review Article

Issam A. Awad and Sean P. Polster

Cavernous angioma (CA) is also known as cavernoma, cavernous hemangioma, and cerebral cavernous malformation (CCM) (National Library of Medicine Medical Subject heading unique ID D006392). In its sporadic form, CA occurs as a solitary hemorrhagic vascular lesion or as clustered lesions associated with a developmental venous anomaly. In its autosomal dominant familial form (Online Mendelian Inheritance in Man #116860), CA is caused by a heterozygous germline loss-of-function mutation in one of three genes—CCM1/KRIT1, CCM2/Malcavernin, and CCM3/PDCD10—causing multifocal lesions throughout the brain and spinal cord.

In this paper, the authors review the cardinal features of CA’s disease pathology and clinical radiological features. They summarize key aspects of CA’s natural history and broad elements of evidence-based management guidelines, including surgery. The authors also discuss evidence of similar genetic defects in sporadic and familial lesions, consequences of CCM gene loss in different tissues at various stages of development, and implications regarding the pathobiology of CAs.

The concept of CA with symptomatic hemorrhage (CASH) is presented as well as its relevance to clinical care and research in the field. Pathobiological mechanisms related to CA include inflammation and immune-mediated processes, angiogenesis and vascular permeability, microbiome driven factors, and lesional anticoagulant domains. These mechanisms have motivated the development of imaging and plasma biomarkers of relevant disease behavior and promising therapeutic targets.

The spectrum of discoveries about CA and their implications endorse CA as a paradigm for deconstructing a neurosurgical disease.

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Sean P. Polster, Hussein A. Zeineddine, Joseph Baron, Seon-Kyu Lee and Issam A. Awad


Cranial dural arteriovenous fistulas (DAVFs) have been associated with dural sinus occlusion, and previous reports have suggested the association of hypercoagulability with some cases. But the prevalence of a hypercoagulable state has not been systematically analyzed in conjunction with laboratory markers and clinical manifestations, including history of thromboembolism or systemic malignancy. The authors hypothesize that laboratory or clinical evidence of a hypercoagulable state, including cancer, is commonly identifiable in consecutively identified patients with DAVFs, with implications for clinical management.


The retrospective cohort study included all patients older than 17 years with cranial DAVFs diagnosed at University of Chicago Medicine during a 6-year period, whose medical records and imaging results were reviewed for objective laboratory or clinical evidence of a hypercoagulable state, including malignancy. Each case was analyzed for implications on clinical management. Data were analyzed in relation to a systematic review of the literature on this association.


Fifteen (88%) of 17 cases of DAVFs had laboratory (n = 8) or clinical evidence of a hypercoagulable state (thromboembolism [n = 8] or cancer [n = 6]). This hypercoagulability or cancer impacted clinical care in all 15 cases.


An underlying hypercoagulable state manifested by laboratory testing or clinically, including cancer, is staggeringly common. It is important to recognize this association, along with its impact on the management of the DAVFs and systemic diseases.