Tag: Cavernous

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Guidelines for the Diagnosis and Clinical Management of Cavernous Malformations of the Brain and Spinal Cord

Neurosurgery 98:3–22, 2026

These guidelines present updated, evidence-based recommendations for diagnosing and managing cavernous malformations (CMs) of the brain and spinal cord, produced by an expert multidisciplinary panel convened by the Alliance to Cure Cavernous Malformation. The document summarizes systematic literature review methods, evidence ratings, and 53 consensus recommendations across epidemiology, genetics, imaging, neurosurgery, and neurology.

Key clinical guidance addresses diagnostic MRI protocols, genetic testing for familial CM (KRIT1/CCM2/PDCD10), surgical/radiosurgical indications including pediatric and geriatric considerations, seizure and headache management, and lifestyle and medication factors potentially affecting hemorrhage risk. Recommendations highlight evidence limitations and prioritize further research.

Diagnosis: MRI with susceptibility-weighted sequences is the gold standard for diagnosing cavernous malformations (CMs); CT may be used in emergencies, but MRI is preferred for follow-up and detailed assessment.

Genetic Testing: Genetic testing for KRIT1 (CCM1), CCM2, and PDCD10 (CCM3) is recommended for individuals with multiple CMs, a family history, or suspected familial CM (FCM); founder mutations exist in certain populations.

Hemorrhage Risk: Annual risk of symptomatic intracranial hemorrhage (ICH) from CMs ranges from 0.7% to 7.5%, with higher risk for recurrent bleeds and in familial cases, especially with CCM3 mutations.

Surgical Management: Surgery is generally reserved for symptomatic, accessible CMs or those causing drug-resistant epilepsy; conservative management is preferred for asymptomatic or deep/eloquent area lesions unless recurrent bleeds occur.

Medical Management: Antiseizure medication is recommended after a first CM-related seizure; standard migraine therapy applies for nonhemorrhagic CMs, and nonaspirin NSAIDs can be used cautiously.

Pregnancy: Pregnancy does not increase the risk of CM hemorrhage compared to nonpregnant states; MRI without contrast is advised for new neurological symptoms during pregnancy.

Medication Risks: Antithrombotic agents (e.g., aspirin) do not increase and may actually lower CM hemorrhage risk, while female hormones may increase risk; thrombolytic use remains controversial and should be considered on a case-by-case basis.

Lifestyle and Emerging Therapies: Vitamin D supplementation, aerobic activity, and a diet low in processed foods are reasonable lifestyle recommendations; propranolol and statins show potential but lack definitive evidence for reducing hemorrhage risk.

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Cavernous angiomas: deconstructing a neurosurgical disease

J Neurosurg 131:1–13, 2019

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 germ-line 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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Volumetric tumor growth rates of meningiomas involving the intracranial venous sinuses

Acta Neurochirurgica (2018) 160:1531–1538

There is currently no consensus as to whether meningiomas located inside the venous sinuses should be aggressively or conservatively treated. The goals of this study were to identify how sinus-invading meningiomas grow, report and compare growth rates of tumor components inside and outside the different venous sinuses, identify risk factors associated with increased tumor growth, and determine the effects of the extent of tumor resection on recurrence for meningiomas that invade the dural venous sinuses.

Methods Adult patients who underwent primary, non-biopsy resection of a WHO grade 1 meningioma invading the dural venous sinuses at a tertiary care institution between 2007 and 2015 were retrospectively reviewed. Rates of tumor growth were fit to several growth models to evaluate the most accurate model. Cohen’s d analysis was used to identify associations with increased growth of tumor in the venous sinuses. Logistic regression was used to compare extent of resection with recurrence.

Results Of the 68 patients included in the study, 34 patients had postoperative residual tumors in the venous sinuses that were measured over time. The growth model that best fit the growth of intrasinus meningiomas was the Gompertzian growth model (r2 = 0.93). The annual growth rate of meningiomas inside the sinuses was 7.3%, compared to extrasinus tumors with 13.6% growth per year. The only factor significantly associated with increased tumor growth in sinuses was preoperative embolization (effect sizes (ES) [95% CI], 1.874 [7.633–46.735], p = 0.008).

Conclusions This study shows that meningiomas involving the venous sinuses have a Gompertzian-type growth with early exponential growth followed by a slower growth rate that plateaus when they reach a certain size. Overall, the growth rate of the intrasinus portion is low (7.3%), which is half of the reported growth rates for other studies involving primarily extrasinus tumors.

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